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Volume 19, Issue 4, Pages 545-716 (November 2003)

The Upper Limb in Cerebral Palsy


Edited by Michael A. Tonkin
articles 1 - 20
1 fmv-viii: TOC
Pages v-viii

2 fmix: forthcoming issues


Page ix

3 The upper limb in cerebral palsy


Page xi
Michael A. Tonkin

4 Introduction
Page 545
Michael A. Tonkin

5 Etiology of cerebral palsy


Pages 547-556
Richard D. Lawson and Nadia Badawi

6 General assessment of the upper limb


Pages 557-564
Caroline Leclercq

7 Functional assessment aided by motion laboratory studies


Pages 565-571
Ann E. Van Heest

8 Casting, splinting, and physical and occupational therapy of hand deformity and
dysfunction in cerebral palsy
Pages 573-584
Judith Wilton

9 Pharmacologic management of the spastic and dystonic upper limb in children


with cerebral palsy
Pages 585-589
Stephen O'Flaherty and Mary-Clare Waugh

10 Botulinum toxin A in the management of upper limb spasticity in cerebral palsy


Pages 591-600
Terence Y. P. Chin and H. Kerr Graham

11 Electrical stimulation in the management of spastic deformity


Pages 601-606
Luis R. Scheker and Kagan Ozer
12 Introduction: Part 2: surgical management
Page 607
Michael A. Tonkin

13 Surgical management of the hand in infantile spastic hemiplegia


Pages 609-629
Eduardo A. Zancolli

14 The upper limb in cerebral palsy: surgical management of shoulder and elbow
deformities
Pages 631-648
A. Landi, S. Cavazza, G. Caserta, A. Leti Acciaro, S. Sartini, M. C. Gagliano
and M. Manca

15 Surgical management of forearm pronation


Pages 649-655
Claudia R. Gschwind

16 Surgical management of wrist and finger deformity


Pages 657-665
Ann E. Van Heest

17 Surgical management of the thumb in cerebral palsy


Pages 667-677
Richard D. Lawson and Michael A. Tonkin

18 Functional and cosmetic outcome of surgery for cerebral palsy in the upper limb
Pages 679-686
Bruce R. Johnstone, Philip W. F. Richardson, Christopher J. Coombs and Josie
A. Duncan

19 Brachial plexus dorsal rhizotomy in hemiplegic cerebral palsy


Pages 687-699
Jayme Augusto Bertelli, Marcos Flávio Ghizoni, Thatiana Rodrigues Frasson
and Karla Samara Fernandes Borges

20 Cumulative Index
Pages 701-716
THE UPPER LIMB IN CEREBRAL PALSY

CONTENTS

Preface xi
Michael A. Tonkin

Part 1: Assessment and Nonsurgical Management

Introduction 545
Michael A. Tonkin

Etiology of Cerebral Palsy 547


Richard D. Lawson and Nadia Badawi
Cerebral palsy has a complex and multifactorial etiology, with perinatal ischemic
hypoxia responsible for only approximately 10% of cases. Preconceptional, antenatal, peri-
natal, and postneonatal influences can interact to result in permanent damage to the de-
veloping central nervous system. This article examines the epidemiology of the condition
and discusses the risk factors and causal pathways that have been defined for cerebral
palsy. It goes on to describe preventative measures and potential areas of intervention.

General Assessment of the Upper Limb 557


Caroline Leclercq
Cerebral palsy is a general term that includes all the sequellae of infantile encephalopa-
thies. This article discusses only infantile cerebral hemiplegia because it is the only one in
which surgery can regularly improve function of the upper limb. Clinical examination of
a spastic patient is lengthy and somewhat difficult, and is best performed together with
all the practitioners involved in the child’s care. Surgery should be aimed at correcting
spasticity, muscle contracture and motor deficit, therefore all these elements must be
specifically assessed during clinical examination.

Functional Assessment Aided by Motion Laboratory Studies 565


Ann E. Van Heest
Preoperative assessment of the patient with cerebral palsy includes assessment of their
static deformity (contractures), dynamic deformity (spasticity versus motor control),
and their sensibility, in order to integrate a treatment plan based on their age, motiva-
tion, and use of the upper limb. Motion laboratory analysis is discussed as a tool that
can assist the surgeon in assessment of patterns of spasticity or control in muscles
considered for tendon transfer or lengthening.

VOLUME 19 Æ NUMBER 4 Æ NOVEMBER 2003 v


Casting, Splinting, and Physical and Occupational Therapy of Hand
Deformity and Dysfunction in Cerebral Palsy 573
Judith Wilton
Facilitation of hand function is a major focus of physical and occupational therapy for
persons with cerebral palsy. A model for analysis of deformities of the hypertonic hand
is presented. It encompasses analysis of movement patterns and associated contracture,
impact of deformity on functional pinch and grip, and discussion of appropriate treat-
ment, including splinting and casting.

Pharmacologic Management of the Spastic and Dystonic Upper Limb in


Children with Cerebral Palsy 585
Stephen O’Flaherty and Mary-Clare Waugh
Pharmacologic management in cerebral palsy may be directed to spasticity, dystonia, or
a combination of these after an assessment of treatment goals. The most common phar-
macologic agents are botulinum toxin (discussed elsewhere) for spasticity and dystonia,
baclofen, diazepam, dantrolene sodium, and tizanidine for spasticity, and L-Dopa and
benzhexol for dystonia. Medications are introduced slowly to monitor efficacy and side
effects and are performed largely on an open trial basis, as evidence of specific treatment
programs is lacking.

Botulinum Toxin A in the Management of Upper Limb Spasticity in


Cerebral Palsy 591
Terence Y.P. Chin and H. Kerr Graham
Botulinum Toxin A has been used in the management of focal dystonia for more than 20
years and in that of focal spasticity for more than 10 years. There have been many open
label studies and randomized clinical trials demonstrating efficacy and safety in the
management of lower limb spasticity in children with cerebral palsy, especially dynamic
equinus (toe walking). In children with juvenile cerebral palsy (JCP), there are good
grounds to suggest that Botulinum Toxin A may be more beneficial in the upper limb
than in the lower limb and further clinical trials are warranted. Botulinum Toxin A
has not been approved for management of upper limb spasticity in children in any coun-
try. All such use is ‘‘off label’’ and should be undertaken only in the context of clinical
trials. Botulinum Toxin A therapy has several upper limb indications and should be con-
sidered complementary to such interventions as occupational therapy, splinting, casting,
and muscle-tendon surgery.

Electrical Stimulation in the Management of Spastic Deformity 601


Luis R. Scheker and Kagan Ozer
This article outlines a nonsurgical approach that includes neuromuscular electrical stim-
ulation and dynamic bracing for the management of spastic deformity in cerebral palsy.
Neuromuscular electrical stimulation is used commonly for lower extremity spasticity.
Its clinical application in upper extremity spasticity, together with dynamic bracing,
is a new entity providing predictable and quick short-term results with significant
improvement in quality of life.

Part 2: Surgical Management

Introduction 607
Michael A. Tonkin

vi CONTENTS
Surgical Management of the Hand in Infantile Spastic Hemiplegia 609
Eduardo A. Zancolli
Upper limb surgery in cerebral palsy is efficacious for patients with a spastic or predom-
inantly spastic pattern of deformity. The deformity can be classified into groups I, IIA,
IIB, and III, according to the ability to extend the fingers with altered wrist position
and the presence or absence of active wrist extension. This scenario allows surgical plan-
ning, following clinical assessment, with selection of appropriate muscle releases, tendon
transfers, and joint stabilizations that are directed to specific clinical presentations. In 47
patients, outcomes were satisfactory in more than 90% of patients in groups I and II, but
were less successful in group III patients.

The Upper Limb in Cerebral Palsy: Surgical Management of Shoulder and


Elbow Deformities 631
A. Landi, S. Cavazza, G. Caserta, A. Leti Acciaro, S. Sartini,
M.C. Gagliano, and M. Manca
The authors report a simple chart that offers a comprehensive picture of spasticity of the
upper limb and provides a more objective method of recording data. Distinction is made
between fixed postures and the residual active range of motion at the shoulder and el-
bow. The presence and function of the muscles can be identified easily on dynamic EMG
studies, which are essential for understanding the degree of spasticity and dyssynergy
related to a single muscle. When spasticity of the upper arm is managed with a global
approach and objectives are defined clearly in advance with the patient and caregivers,
treatment of shoulder and elbow deformities can achieve important results for personal
hygiene or functional targets.

Surgical Management of Forearm Pronation 649


Claudia R. Gschwind
Pronation deformity is part of the common deformity pattern in cerebral palsy. A clas-
sification and treatment plan is proposed. Specific assessment of forearm rotation in con-
junction with assessment of elbow, wrist, and finger deformity is recommended to
optimize surgical planning and to avoid over or under correction of the pronation
deformity.

Surgical Management of Wrist and Finger Deformity 657


Ann E. Van Heest
Children with cerebral palsy can improve their motor function and perhaps also their
sensibility function with appropriately planned and executed tendon release and transfer
surgery. Balance of the wrist and fingers is the key element in improvement of upper
limb function.

Surgical Management of the Thumb in Cerebral Palsy 667


Richard D. Lawson and Michael A. Tonkin
The typical thumb deformities in cerebral palsy are adduction contracture and adduc-
tion-flexion contracture (thumb-in-palm deformity). Surgical treatment of thumb defor-
mity involves a rebalance of forces acting across the thumb joints by release of spastic
muscles and augmentation of paretic muscles. Unstable joints may need stabilization
or arthrodesis.

CONTENTS vii
Functional and Cosmetic Outcome of Surgery for Cerebral Palsy in
the Upper Limb 679
Bruce R. Johnstone, Philip W.F. Richardson, Christopher J. Coombs, and
Josie A. Duncan
Surgical treatment of the upper limb in cerebral palsy aims to improve function and
cosmesis in many patients and ease of dressing and hygiene in severely disabled pa-
tients. This has been achieved through multiple simultaneous procedures. These proce-
dures include the release, lengthening, or paralysis of deforming spastic muscles, tendon
transfers, and joint stabilizations. The authors present an approach to the surgical man-
agement of upper limb cerebral palsy and a patient/caregiver-based outcomes assess-
ment. In addition to the functional scale developed by House, cosmesis, dressing,
hygiene, and overall satisfaction have been assessed and found to be significantly
improved.

Brachial Plexus Dorsal Rhizotomy in Hemiplegic Cerebral Palsy 687


Jayme Augusto Bertelli, Marcos Flávio Ghizoni,
Thatiana Rodrigues Frasson, and Karla Samara Fernandes Borges
Brachial plexus dorsal rhizotomy interrupts pathologic afferents, leading to high resolu-
tion of spasticity and general upper limb functional improvement. The authors present
a cohort clinical study with 20 patients and discuss the indications, hand sensibility
preservation, and motor control after dorsal rhizotomy.

Cumulative Index 2003 701

viii CONTENTS
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Hand Clin 19 (2003) xi

Preface

The upper limb in cerebral palsy

Michael A. Tonkin, MD, FRACS


Guest Editor

This issue of the Hand Clinics is devoted to the of acting as a guest editor is to appreciate the charm
upper limb in cerebral palsy. Surgery undoubtedly of the English language as written by those for
has a role to play—more so for disorders in which whom English is not the first language. I have tried
spasticity is dominant—and perhaps, as far as to retain the flavor of nationality in English expres-
function is concerned, is ideally directed toward sion, because I believe it enriches us all.
the child with spastic hemiplegia who has good The faults of any multi-authored book are
sensibility and pre-existing function in the affected repetition and lack of continuity. I have attempted
upper limb. Surgery for severe deformity, with or to minimize repetition while allowing each article
without pain, is beneficial in improving both to stand on its own as a reference for its particular
appearance and ease of management for carers. topic.
In severely affected cases, function is unlikely to Finally, this issue would never have been pub-
be improved. lished were it not for the invaluable assistance of
Surgery is not suitable for all and should only Susan Filan, research assistant in the Department
be undertaken after careful and frequent consid- of Hand Surgery at the Royal North Shore Hospi-
eration. Therefore, Part 1 of this issue is devoted tal in Sydney, in whose hands I placed most of the
to assessment and nonsurgical management so onerous tasks associated with preparing these 14
that the surgeon to whom this text is directed articles for publication. I have no doubt that the
has the benefit of the assessment and management invited authors are aware of her efforts.
methods provided by nonsurgical physicians and My gratitude also goes to Deb Dellapena at
therapists, who play such an important role in Elsevier, who provided invaluable advice and sup-
the care of those afflicted by cerebral palsy. port to the guest editor and the authors.
The authors are not only of disparate medical
background but also reflect international knowl-
Michael A. Tonkin, MD, FRACS
edge and experience in this field, with European,
Department of Hand Surgery
North American, South American, and Australian
Royal North Shore Hospital
contributors. If there is a preponderance of Austra-
Pacific Highway
lian authors, this is because the guest editor was
St. Leonards, NSW 2065, Australia
able to exert greater editorial control over his own
countrymen and -women. However, one of the joys E-mail address: mtonkin@surgery.usyd.edu.au

1042-3680/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00097-0
Hand Clin 19 (2003) 545

Part 1: assessment and nonsurgical management


Introduction
Michael A. Tonkin, MD, FRACS
Department of Hand and Peripheral Nerve Surgery, Royal North Shore Hospital,
St. Leonards, NSW 2065, Australia

This section provides the reader with an before surgery is considered. These rely very much
understanding of the causes of cerebral palsy, on the expertise of general physicians, neuro-
how to assess the overall conditions affecting the logists, therapists, caregivers and parents. Wilton
afflicted individual, and the nonsurgical manage- provides the reader with a detailed account of the
ment protocols, which include physical therapy therapist’s classification methods and management
and splinting, pharmacological management, the protocols. O’Flaherty and Waugh summarize the
administration of botulinum toxin, and the use of medications available to control both spasticity
electrical stimulation. and dystonia. Graham’s unit is at the forefront of
The article by Lawson and Badawi provides an investigations into the efficacy of botulinum toxin
insight into the multiplicity of pathways that may administration. Finally, the Louisville group have
lead to a diagnosis of cerebral palsy, dispelling the described their experience with a combination of
previously commonly held theory of anoxia at electrical stimulation and splinting.
birth as the culprit in causation. The assessment As a unit, these articles provide great insight
articles by Leclerq and Van Heest give an for the surgeon who must not undertake treat-
indication of the complex reviews undertaken ment in isolation.

E-mail address: mtonkin@surgery.usyd.edu.au

0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00095-7
Hand Clin 19 (2003) 547–556

Etiology of cerebral palsy


Richard D. Lawson, MB, BS, FRACSa,1,
Nadia Badawi, PhD, FRACPb,c,*
a
Department of Orthopaedic Surgery, The Children’s Hospital at Westmead, New South Wales, Australia
b
Department of Neonatology, The Children’s Hospital at Westmead, Locked Bag 4001, Parramatta,
New South Wales, NSW 2145, Australia
c
University of Sydney, New South Wales, Australia

Cerebral palsy refers to a group of disorders approximately 1 in 20 survivors, or 50 times as high.


of the central nervous system characterized by Although they carry a much lower individual risk,
aberrant control of movement or posture, present infants born at or near term account for at least half
since early in life and not the result of recognized of all cases of cerebral palsy [9]. Continuing
progressive disease. The motor abnormalities that improvements in obstetric and neonatal care, which
define cerebral palsy often are accompanied by have resulted in significant reductions in intra-
other neurologic problems, including cognitive partum hypoxia-related perinatal death, have not
impairment, seizure disorders, and impairments in been associated with any consistent decrease in the
vision, hearing, or speech [1–5]. rate of cerebral palsy in term infants [10].
Little first attributed cerebral palsy to a difficult
birth in 1843 and this view remained current for
Etiology of cerebral palsy
nearly 150 years [6]. Current thinking implicates
perinatal asphyxia in less than 5%–10% of cases. Many risk factors for cerebral palsy have been
The alternative view proposed by Freud, that described in the preconceptional, antepartum,
cerebral palsy could be a manifestation of ab- intrapartum, and postneonatal periods. Because
normal fetal development [7], now is given more the final presentation may be the same regardless of
emphasis. Most children with cerebral palsy are when the damage occurred, however, the etiology
born following uncomplicated pregnancies and in any given case is often difficult to ascertain [11].
normal deliveries, with no evidence of fetal dis-
tress or immediate neurologic impairment [8]. Preconception risk factors
The prevalence of cerebral palsy is approxi- Some maternal conditions are associated with
mately 1.0–2.5 per 1000 live births and is strongly a significantly greater risk for cerebral palsy in the
influenced by gestational age and birthweight. offspring.
Although cerebral palsy occurs in less than 1 per
1000 survivors for whom the birthweight was Neurologic disorders
>2500 g, in early preterm children this rate is Women with seizure disorders are at increased
risk for cerebral palsy, stillbirth, microcephaly,
intellectual disability, and seizures in their off-
1
Present address: Discipline of Pediatrics and Child spring. Maternal intellectual disability and neu-
Health, University of Sydney, New South Wales, NSW rologic and neuromuscular disorders also are
2145, Australia. associated with neurologic abnormality in the
* Corresponding author. Department of Neonato- offspring, including cerebral palsy [9,12–14].
logy, The Children’s Hospital at Westmead, Locked Bag
4001, Parramatta, New South Wales, NSW 2145, Infertility treatment
Australia. There is evidence to suggest an increased risk
E-mail address: nadiab@chw.edu.au (N. Badawi). for cerebral palsy associated with in vitro
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00040-4
548 R.D. Lawson, N. Badawi / Hand Clin 19 (2003) 547–556

fertilization (IVF) [15]. Stromberg et al per- Antepartum risk factors


formed a population-based retrospective cohort
Other antepartum maternal medical conditions
study in which the investigators compared
that are associated strongly with cerebral palsy
neurologic problems in 5680 children born after
include severe pre-eclampsia and bleeding in
IVF, with 11,360 matched controls. The most
pregnancy (particularly placental abruption and
common neurologic diagnosis was cerebral palsy,
placenta previa) [30,31]. The mechanism of injury
for which children born after IVF had an overall
of these conditions is uncertain but may be
increased relative risk of 3.7 (2.0–6.6). These risks
mediated in some situations through antepartum
were mostly because of the high frequency of
fetal hypoxia. Placental pathology and polyhy-
twin pregnancies, low birthweight, and prematu-
dramnios also are associated with an increased
rity in this group. Even IVF singletons have
risk for cerebral palsy [32].
a relative risk for cerebral palsy of 2.8 (1.3–5.8),
however, compared with non-IVF singletons [16].
Antepartum infection
To limit these risks, the authors recommend that
There is increasing interest in the role of
only one embryo should be transferred during
infection in the etiology of fetal brain pathology
IVF.
[33]. Damaging systemic infections, including
It is of interest in this context that a positive
toxoplasmosis, rubella, cytomegalovirus, and lis-
association also has been noted between cerebral
teriosis [9] are well recognized and attention is being
palsy and abnormally long or short menstrual
focused now on the role of ascending genitourinary
cycles, intervals of more than 3 years or less than 3
infection. In term or near-term infants, markers of
months between pregnancies, and previous spon-
infection are associated with an estimated nine-fold
taneous abortion and still birth [17].
increase in the risk for spastic cerebral palsy [34].
Intrauterine infection is also an important
cause of preterm birth. Many studies in preterm
Thyroid disease
infants have found markers of infection to be
Thyroid disease in the mother has been
associated with cerebral palsy. Evidence of in-
associated consistently with an increased risk for
fection or inflammation is so common in preterm
cerebral palsy in the infant. Other adverse
birth that it is hard to separate its contribution
neurologic outcomes including schizophrenia
from the effect of prematurity itself [35–38].
and suboptimal neuropsychologic development
[18–20] also have been described. Follow-up data
Pre-eclampsia
from the Australian Collaborative Trial of Ante-
Maternal pre-eclampsia is a consistent risk
natal Thyrotropin Releasing Hormone have
factor for cerebral palsy in term infants. Severe
suggested a link between the administration of
pre-eclampsia was associated strongly with term
antenatal thyrotropin-releasing hormone and de-
newborn encephalopathy in a population-based
velopmental delay at 1 year of age [21].
study [27]. There has been recent controversy
This link is biologically plausible. Thyroid
about the role of pre-eclampsia in preterm births,
hormones and iodine play an essential role in
however. In these infants, cerebral palsy was less
the development of the nervous system, including
common in births following pre-eclampsia than in
cerebral neurogenesis and migration and myelo-
other causes of preterm birth. There are several
genesis [22,23]. Because the fetal thyroid does not
possible reasons for this, including the planned
produce substantial amounts of thyroxine until
nature of most preterm births complicated by pre-
mid gestation [24], it is possible that relative
eclampsia, which allows time for steroids and
under-replacement in hypothyroid women may
appropriate in utero transfer, and the potentially
compromise the amount of thyroid hormone
more damaging nature of many other precursors
reaching the fetus at crucial stages of early
of preterm birth, such as abruption, chorioam-
neurologic development. Severe iodine deficiency
nionitis, and cord complications [39,40].
resulted in a cluster of spastic diplegia and deaf
mutism in Papua New Guinea [25].
Infant characteristics associated with
Thyroid disease in pregnancy is one of the
cerebral palsy
strongest independent risk factors for newborn
encephalopathy with an odds ratio of 9.7 (95%CI, Gestational age
1.97–47.91) [26–28]. Newborn encephalopathy Animal studies have shown that the post-term
often precedes cerebral palsy [29]. fetus is more vulnerable to asphyxia, a finding of
R.D. Lawson, N. Badawi / Hand Clin 19 (2003) 547–556 549

particular significance in the context of the recent When one twin dies in utero, the risk for cerebral
debate about the merits of induction at 41 weeks’ palsy is increased markedly in the surviving twin.
gestation [41,42]. The capacity of the fetus to Multiple gestation seems to be an independent risk
recover from an insult also is believed to be factor for cerebral palsy, with the rate of cerebral
strongly associated with gestational age, with palsy increasing with the number of fetuses.
post-term infants having a lesser capacity for Triplets have a cerebral palsy rate of 28/1000
recovery [42]. survivors, compared with 7.3/1000 in twins and 1.6/
The prevalence of cerebral palsy among pre- 1000 in singletons. This is becoming increasingly
mature infants increased during the 1970s and important with advancing maternal age and in vitro
1980s, although it has stabilized in recent years in fertilization, which are associated with multiple
developed countries. The initial increase may have gestation [49–51].
been related to increasing survival among pre-
mature infants with postnatal damage, whereas Growth restriction
ongoing improvements in perinatal and neonatal In all animal species, survival correlates with
intensive care probably have contributed to the percentile birth weight, with neonatal survival
recent stabilization in rates [9]. greatest among infants whose birth weight is
Cerebral palsy in premature infants currently is slightly greater than the population average [52–
believed related to cerebral ischemia and inflam- 55]. Many investigators have described an associ-
mation. Premature infants are more likely to be ation between intrauterine growth restriction
exposed to events that predispose to systemic (IUGR) and cerebral palsy and newborn seizures
hypotension and also are less able to compensate [56–59]. Intrauterine growth restriction may in-
for these episodes of hypotension because of crease the susceptibility of the fetus to asphyxia
impaired autoregulation. Many investigators have during labor, and these fetuses are also at higher
noted a strong association between cerebral palsy risk for intrauterine death [57,60]. There can be
in premature infants and chorioamnionitis, ante- little doubt that for many growth-restricted infants,
natal infection, and neonatal sepsis [43]. however, cerebral injury has occurred before the
Cytokines produced in response to maternal onset of labor. An insult severe enough to have an
and fetal infection/inflammation can exert direct adverse impact on growth also is capable of
toxic effects on cell function, differentiation, affecting intrauterine cerebral development.
interaction, and indirect effects by way of the Growth restriction thus may have a direct effect
induction of fetal hypotension and disseminated on cerebral development, be an indicator of
intravascular coagulation. Periventricular leuko- a separate damaging event that also affected
malacia is a common finding in patients with growth, or be part of a syndrome that affects
cerebral palsy and is associated strongly with fetal growth and cerebral function.
sepsis and elevated levels of inflammatory cyto- IUGR also is predictive of other subsequent
kines (IL-6 and TNF-a) in the blood and amniotic neurodevelopmental disability, such as global
fluid of preterm infants [44,45]. These inflamma- developmental delay, learning disability, and
tory mediators may be a final common pathway psychiatric disorders. These sequelae are more
to neurologic injury for a variety of pathogenic frequent in the presence of other associated
disorders. antepartum complications [61,62].
Antenatal steroids are used in women who are
anticipated to go into premature labor to accel- Gender
erate fetal lung maturity. Another benefit of this Many studies have noted the preponderance of
therapy is a reduced risk for cerebral palsy, with males affected by cerebral palsy and intellectual
a 50% reduction in white matter damage and disability [63]. These findings warrant further
a 30% reduction in the risk for cerebral palsy [46]. genetic and biologic investigation, as they may give
Conversely, there is accumulating evidence that clues to causation. Investigation along this line
multiple doses of antenatal steroids and lengthy already has yielded results, with identification of
postnatal exposure to steroids may be harmful to sex-linked conditions such as the Fragile X
neurologic development [47,48]. syndrome. In addition, it has become apparent
that there are differences in myelination rates
Multifetal gestation between male and female fetuses. The significance
Twins are more likely to be born prematurely of this in the etiology of neuropathology has yet to
than singletons and to have lower birth weights. be determined.
550 R.D. Lawson, N. Badawi / Hand Clin 19 (2003) 547–556

Placental pathology cerebral palsy has been mapped to chromosome


Because the placenta is the direct link between 2q24-25 [70].
mother and fetus, the idea that it may yield
important etiologic and prognostic information The thrombophilias
for the management of the sick newborn is an A recent study of children with cerebral palsy
appealing one [64]. demonstrated significant differences in the levels of
Small placental size has been linked with cytokines and autoimmune or coagulation factors
subsequent cerebral palsy, whereas placental in the cord blood of these infants compared with
infarcts have been shown to correlate with control subjects [71]. These findings raise the
prenatal cerebral ischemic lesions in stillborn possibility that abnormalities of coagulation in
infants [65]. In any particular patient, placental the fetus may result in antepartum or perinatal
pathology also may give a clue to the etiology of brain damage. The most common of these disor-
cerebral palsy or encephalopathy by revealing ders is the factor V Leiden mutation, found in
such clues as evidence of sepsis, retroplacental approximately 6% of Caucasian populations.
bleeding, vasculitis in autoimmune diseases, or Alone, the Leiden mutation is associated with
placental vascular malformations [64,66]. Other a low level of risk, but with the addition of other
significant findings include evidence of extensive genetic or acquired risk factors, the risk increases
infarction in conditions such as pre-eclampsia, markedly. Other coagulation disorders may coexist
systemic lupus erythematosus, maternal or fetal in affected individuals, suggesting that several risk
thrombophilias, and cord abnormalities such as factors may need to be present to cause neurologic
true knots. damage. In this study, children with cerebral palsy
who also carried a coagulation disorder were more
Birth defects and genetic factors likely to have additional pregnancy complications
There is a high rate of birth defects among than other children with cerebral palsy. These
children with cerebral palsy and newborn enceph- included pre-eclampsia, fetal growth restriction,
alopathy [67], and abnormalities of the central low Apgar scores, neonatal seizures, and a diag-
nervous system are particularly common. A recent nosis of asphyxia during birth [71,72].
study of Californian infants found that birth The link between thrombophilias and cerebral
defects occurred in 19.2% of children with cerebral palsy may result directly from central nervous
palsy and 4.3% of control children [68]. The high system infarction [73] or indirectly through pla-
rate of birth defects in children with cerebral palsy cental infarction with the consequent release of
has prompted some pediatric neurologists to vasoactive compounds into the fetal circulation.
recommend that patients with neonatal encepha- These findings are particularly exciting because
lopathy should have perinatal MRI of the brain, these conditions are potentially open to therapeu-
which may reveal neurodevelopmental lesions tic intervention and may represent an avoidable
such as cerebral dysgenesis [69]. Non-neurologic pathway to neurologic injury.
birth defects are more common in infants with
The intrapartum period
cerebral palsy. This raises the possibility that an
insult that occurs at a time during which a partic- Labor is the period of most intensive scrutiny
ular organ system is vulnerable, causing maldevel- during pregnancy. There is, however, a wide range
opment of that system, also may be sufficient to of ‘‘expert opinion’’ as to what constitutes the
affect cerebral development or function. This may normal range of labor events. There is now an
occur with or without a recognizable structural expectation by the community that pregnancy will
lesion in the central nervous system. result in a normal outcome. If the course of labor
deviates from a perceived norm, there is often an
Genetic causes assumption that events observed during this time
McHale found that approximately 2% of were causally related to the outcome. This perva-
cerebral palsy cases in Swedish and English sive belief, accurate in only a minority of cases, has
populations were attributable directly to genetic had a profound effect on medical practice.
factors. Athetoid and ataxic variants of cerebral Factors in the intrapartum period that have
palsy are particularly likely to have a genetic basis. been studied for association with cerebral palsy
Inheritance is typically autosomal recessive, but include intrapartum fever, acute intrapartum
autosomal dominant and sex-linked syndromes events, fetal malposition, the mode of delivery,
have been noted. A gene for symmetric spastic meconium-stained liquor, and hypoxia.
R.D. Lawson, N. Badawi / Hand Clin 19 (2003) 547–556 551

Intrapartum fever Mode of delivery


Maternal pyrexia in labor is associated with Caesarean section does not seem to decrease
a significantly increased risk for cerebral palsy in the prevalence of cerebral palsy in fetuses judged
infants with a birthweight >2500g (adjusted OR to be at risk from abnormalities in fetal heart rate
3.3; 95% CI, 1.4, 8.1) [34]. Fever may represent monitoring [76]. Cerebral palsy rates are consis-
infection of mother or infant, but can occur in the tent across countries with westernized medical
absence of an obvious source or identified organ- systems despite marked disparities in the rate of
ism. The association with cerebral palsy may be caesarean section.
mediated directly by fetal infection or indirectly
through inflammatory cytokines that cause the The contribution of possible intrapartum hypoxia
fever. In animal studies, fever per se, without Fetal hypoxia of some degree is common, and
underlying infection, has been associated with the perhaps even normal in labor, yet most asphyxi-
development of metabolic acidosis and hypoxia ated infants do not develop cerebral palsy [77]. It is
[74]. not known what represents excessive exposure to
hypoxia. Long-term follow-up studies linking
Acute intrapartum events oxygen saturations and neurologic outcomes are
Labor is an inherently unstable condition, with not available, and at present we have no means of
the fetus called on to survive substantial periods adequately assessing individual susceptibility [78].
of hypoxia and ischemia as part of the normal With currently used methods of monitoring, it is
course of labor. The fetus has many mechanisms difficult to meaningfully define clinically significant
to protect itself in these circumstances, such as the intrapartum hypoxia except in severe cases [79].
capacity to extract oxygen from maternal blood In practice the situation is further complicated.
at low partial pressures, redundancies in placental The traditional markers of intrapartum hypoxia
surface area for diffusion, and the capacity to that include low Apgar scores, the passage of
redirect cardiac output to the cerebral circulation meconium, and abnormalities of fetal heart rate
to protect the brain. The result is that the fetal have poor specificity. Moreover, none of the
central nervous system is able to tolerate low current indicators of asphyxia give reliable in-
partial pressures of oxygen for extended periods formation about the timing or duration of an
of time [75]. There are occasions, however, in asphyxial insult, which can occur before labor and
which catastrophic events can overwhelm the be acute or chronic. The mere presence of
fetus’ protections. These include placental abrup- intrapartum hypoxia is insufficient to demonstrate
tion, cord complications (eg, prolapse or tighten- a causal link between that hypoxia and the
ing of true knots), uterine rupture, or hypertonic observed outcome. For many infants who later
uterine contractions. In these circumstances even show signs of injury, this hypoxia may be ‘‘an
a well nourished fetus may sustain damage. In innocent bystander’’ unrelated to outcome. It also
a fetus with an underlying compromise, the may contribute proportionally in the context of
reserve to cope with normal labor or its compli- prior compromise, with pre-existent vulnerability
cations may be diminished greatly. It seems clear sensitizing the fetus to what otherwise may have
that some fetuses, because of good reserve, been a nondamaging event [78,80].
tolerate even severe complications and emerge Fetal intrapartum hypoxia currently is believed
unscathed, whereas in others even normal labor to be causally involved in up to 10% of cases of
may cause or exacerbate damage. Though this cerebral palsy, and its significance lies in that it
subgroup of cerebral palsy may be fairly small, it may be potentially avoidable. It is important to
is of vital importance because it is potentially remember, however, that even when intrapartum
avoidable. hypoxia plays a primary role, it is not always
avoidable, nor necessarily evidence of incorrect
Malposition management. In an attempt to identify those cases
Malposition is a common complication of in which intrapartum hypoxia may have had a role
pregnancy and labor. Although it is known that in causation, International Consensus Guidelines
nonvertex fetal presentations are associated with were published in 1999 to define the criteria
cerebral palsy, it remains uncertain whether this necessary to attribute a neurologic deficit to acute
relationship is causal, as the abnormal lie may perinatal hypoxia [75]. The guidelines suggest that
itself be a manifestation of pre-existent fetal three factors are essential if a perinatal hypoxic
hypotonia [17,76]. event is to be considered as potentially causative:
552 R.D. Lawson, N. Badawi / Hand Clin 19 (2003) 547–556

metabolic acidosis, the presence of neonatal [39]. Several international multicenter trials cur-
encephalopathy, and cerebral palsy of the spastic rently are examining this possibility.
quadriplegic or dyskinetic type. Further guidelines
Dexamethasone
on factors that supported an intrapartum hypoxic
Though there is evidence that the antenatal use
origin and those that made it less likely also were
of fluorinated corticosteroids such as Celestone
included.
and dexamethasone may be protective against
Kernicterus cerebral palsy, prolonged neonatal use may be
In the past, Rhesus isoimmunization was associated with an increased risk for cerebral palsy.
a major cause of hemolytic disease of the newborn, The timing and duration of treatment is yet to be
which can result in significant deposits of bilirubin determined but is crucial, for what may be
in the developing brain, a potent cause of cerebral protective in one period may well be harmful in
damage and subsequent kernicterus. The advent of another.
anti D has reduced markedly the burden of Rhesus
Fetal intrapartum monitoring
disease, although there have been several reports
of kernicterus in the United States, possibly related It was hoped that the introduction of cardio-
to early discharge programs [81]. tocograms would lead to a decrease in the rate of
cerebral palsy, but this has not occurred. In-
Postneonatally-acquired cerebral palsy
terpretation of fetal heart rates is difficult, with
Postneonatal factors, such as meningoenceph- a wide interobserver variability. The clinical
alitis, head injury, cerebrovascular accidents, near response to various patterns also has been shown
drowning, and nonaccidental injury now are to be inconsistent [76]. Cardiotocographs lack
believed to account for 15% of cases of cerebral specificity, with up to 79% of tracings having
palsy, a proportion that seems to be increasing in nonreassuring patterns [75]. The features that are
recent years [9]. believed to indicate possible fetal compromise,
such as late decelerations in the fetal heart rate
Prevention after the onset of contractions and diminished
beat-to-beat variability, have a false positive rate
Medical management of 99.7%. Intervention in the form of caesarean
Infection, autoimmunity, coagulation, and section on the basis of a nonreassuring fetal heart
thyroid disorders are problems for which modern rate pattern does not seem to reduce the risk for
medicine has therapeutic tools; however, screen- cerebral palsy [84].
ing and management strategies that optimize In a randomized, controlled trial of electronic
neurologic development of the fetus or neonate fetal monitoring in labor by MacDonald and
in these circumstances remain to be identified. The colleagues, infants who had been monitored
potential benefits of any intervention must be electronically in labor were less likely to have
balanced carefully against potential risks in any neonatal seizures than those who had intermittent
new public health policy [82]. auscultation [85], but there was no increased risk
There may be novel targets for therapy. For for cerebral palsy in the intermittent auscultation
example, if certain cytokines are neurotoxic, then group at follow-up. New methods of monitoring
anticytokine therapies may be possible if at-risk are being developed, including continuous fetal
infants can be identified reliably. Use of anti- oxygen monitoring and computerized cardiotoco-
cytokines requires great caution, however, because graph interpretation systems. It is vital that these
of the important roles some of the cytokines play systems are validated in their predictive power for
in normal brain development, in limiting injury to an abnormal outcome before being accepted into
cerebral tissue and in immunologic defense [83]. clinical practice.

Magnesium sulfate
Summary
Some studies have demonstrated a decrease in
the incidence of cerebral palsy in the children of Cerebral palsy has a complex and multifacto-
mothers treated for pre-eclampsia, and this has rial etiology. Approximately 5%–10% of cases can
stimulated research into possible protective effects be ascribed to perinatal hypoxia, but the vast
of magnesium sulfate, an agent commonly used in majority of cases are caused by the interplay of
the treatment of pre-eclampsia in many countries several risk factors and antenatal, perinatal, and
R.D. Lawson, N. Badawi / Hand Clin 19 (2003) 547–556 553

neonatal events. The strongest risk factors include [8] Nelson KB, Ellenberg JH. The asymptomatic
prematurity and low birth weight. The prevalence newborn and risk for cerebral palsy. Am J Dis
of cerebral palsy has remained constant despite Child 1987;141(12):1333–5.
improvements in obstetric and neonatal care. For [9] Stanley F, Blair E, Alberman E. Cerebral palsies:
epidemiology and causal pathways. Clinics in de-
a long time, the only causal factors explored to
velopmental medicine. No. 151. London: MacKeith
account for risk for cerebral palsy were complica-
Press; 2000.
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interacting pathogenic assaults may overwhelm
Education and Welfare; 1972.
natural defenses and produce irreversible brain
[13] Nelson KB, Ellenberg JH. Maternal seizure disorder,
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Hand Clin 19 (2003) 557–564

General assessment of the upper limb


Caroline Leclercq, MD
Institut de la Main, 6 Square Jouvenet, Paris 75016, France
Institut de la Main, Neurological Rehabilitation Centre, Coubert 75016, France

Clinical examination procedures (eg, injections) are necessary, they


must be left for last.
Clinical examination is the key to successful
Symptoms may vary with the child’s emotional
treatment. Unlike many other conditions, there is
state and fatigue level; even the weather can mod-
no standard clinical picture for spastic upper
ify the clinical picture. Moreover, many children,
limbs, whatever their cause, as the condition
especially younger ones, cannot cooperate
primarily depends on the extent of the brain
throughout the entire examination and require
damage, which can vary greatly. A careful
that this examination be fragmented. It is there-
assessment of all parameters is therefore manda-
fore not wise to decide on surgery after a single
tory to get an accurate clinical picture of the
examination, and at least a second session is
deficits and potentials in each patient and to treat
recommended to have a more accurate view of the
them accordingly.
clinical picture. Video recording of this examina-
This examination is performed with a fourfold
tion is most helpful in the decision making and in
goal:
evaluation of surgical outcome.
 Evaluate spasticity
 Evaluate the motor and sensory deficit in the Resting posture of the upper limb
upper limb
Inspecting the limb at rest before examination
 Evaluate the existing function and functional
provides information on the amount of spasticity.
needs of the upper limb
It is done while the child is sitting or lying down
 Perform a complete general examination to
motionless, with his or her attention distracted
seek associated neurologic disorders and seek
elsewhere (eg, TV, movie). If predominant,
contraindications to surgery
spasticity usually leads to a resting posture in
This examination is lengthy and requires shoulder adduction and internal rotation, elbow
detailed knowledge of neurology, pediatrics, and flexion, forearm pronation, and wrist flexion. If it
physiatry. It is best performed with all the is moderate, however, the resting posture may be
specialists involved in the child’s care (physiatrist, normal.
physical therapist, occupational therapist, pedia- The fingers may assume varied positions when
trician, and surgeon). It should be done in a warm, spasticity is predominant. Most often they are
quiet, and friendly environment (eg, toys, adapted curled in a clenched fist, but they also can display
furniture). Trust must be established by the a swan neck deformity or a claw type deformity.
examiner at the beginning of the evaluation; this A boutonniere type of deformity is less common.
is essential because the child’s cooperation is The thumb can assume either an adducted
mandatory for the sensorimotor evaluation and posture or an adducted and flexed posture.
because spasticity may increase considerably if The adducted thumb is often in a slight retropo-
the child is frightened or recalcitrant. If painful sition with the metacarpophalangeal (MP) and
interphalangeal (IP) joints extended. The flexus-
adductus thumb, often referred to as ‘‘thumb-
E-mail address: caroline.leclercq@free.fr in-palm,’’ is embedded in the palm with full
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00059-3
558 C. Leclercq / Hand Clin 19 (2003) 557–564

opposition and full flexion of MP and IP joints. a combination of excessive traction on the
Often the clenched fist is curled around the extensor tendons and paralysis of the intrinsic
thumb. muscles.
Any factor that aggravates spasticity increases Thumb spasticity involves mainly the ad-
these deformities. ductor pollicis, resulting in an adducted posi-
tion. When the flexor pollicis longus (FPL)
Evaluation of spasticity also is involved, a thumb-in-palm deformity
develops.
Spasticity is a muscle hypertonicity defined by
2. It is elastic. Attempts at stretching the
five classic characteristics.
involved muscles meet with resistance, which
1. It is selective, predominant in flexor and increases with the amount of strength applied
adductor muscles, and is responsible for the and which correlates with the degree of spas-
characteristic flexion-pronation deformity of ticity. Unlike plastic contractures, the joint
the upper limb. It is usually less evident in the returns to its initial position as soon as the
shoulder and elbow and more severe in the opposing force is stopped. If the opposing
distal part of the upper limb. force is maintained long enough, however, the
In the shoulder it involves the adductor and muscles usually yield, sometimes abruptly
internal rotator muscles and in the elbow the (this is referred to as the ‘‘penknife blade’’
flexor muscles, predominantly the biceps phenomenon). The degree of resistance to
brachii and the brachialis, but possibly also stretching can be quantified by Ashworth’s
the brachioradialis [1]. The medial epicondy- scale, modified by Bohannon [2].
lar muscles, often spastic, are responsible for 3. It is present at rest and exaggerated with
the pronation deformity and also contribute voluntary movement, emotion, fatigue, and
to the elbow flexion deformity. pain.
The wrist flexors, especially the flexor carpi 4. Osteotendinous reflexes are exaggerated,
ulnaris (FCU), are frequently the muscles brisk, diffuse, and polykinetic. Clonus is
most involved by spasticity in the upper limb, infrequent in the upper limb.
leading to a characteristic hyperflexion and 5. Synkineses may be associated. Synkinesis is
ulnar deviation of the wrist. If the finger the phenomenon wherein paralyzed muscles,
flexors are also spastic, they contribute to this incapable of a certain voluntary movement,
deformity. Spasticity of the finger extrinsic attempt to execute this movement in associ-
muscles is tested by tapping briefly on the ation with contraction of accompanying in-
pulps (flexors) or the nails (extensors), which tact muscles. For example, in Souques
produces an exaggerated response of the synkinesis, the hand displays ‘‘active’’ exten-
tested muscles. sion and abduction of the fingers with
In such cases, spasticity of the intrinsic voluntary shoulder abduction.
muscles of the hand is extremely difficult to
evaluate because of associated deformities. Motor assessment
An intrinsic-plus deformity (MP joint flexion
and IP joint extension) results from spasticity Motor examination of the upper limb is not
of the interossei. A swan neck deformity easy in children, especially in children younger
(proximal interphalangeal [PIP] joint hyper- than 5 years of age. The child should be provided
extension and distal interphalangeal [DIP] with toys of different forms and colors and should
joint flexion) may be the result of either be observed at play [3]. Each muscle or group of
excessive traction on the extensor tendons muscles is evaluated for (1) voluntary motor
caused by excessive wrist flexion (extrinsic control, (2) fibrous contracture, and (3) joint
swan neck) or to spasticity of the interossei range of motion.
muscles (intrinsic swan neck), in which case it
is often associated with MP joint flexion. Voluntary motor control
Passively flexing the wrist usually reduces the The palsy usually predominates in the extensor
intrinsic swan neck, whereas it increases the and supinator muscles and in the distal part of the
extrinsic swan neck. A claw-type deformity upper limb. Rather than a true paralysis, it is
with the MP joints hyperextended and the a deficit in voluntary control linked with the
PIP joints flexed usually is caused by pyramidal tract involvement (‘‘pseudo palsy’’).
C. Leclercq / Hand Clin 19 (2003) 557–564 559

Voluntary motion is often slow and may be better Motor blocks provide several types of in-
in a limited range of motion. formation:
This examination may become difficult when
They differentiate spasticity from fibrous con-
the antagonist muscles are severely spastic.
tracture in flexor, adductor, and pronator
Weakness generally predominates in the distal
muscle groups
muscles spared from spasticity (wrist and finger
They allow assessment of individual muscles
extensors, abductor, and extensor pollicis longus,
after removing spasticity of the antagonists or
and supinator muscles). The proximal muscles
co-contractions of adjacent muscles
(shoulder external rotators and abductors and
Within a spastic muscle group they help
elbow extensors) are involved to a lesser degree.
determine which muscles are spastic and which
The lack of control varies with limb position.
are not
For instance, voluntary movement of the thenar
muscles often is facilitated by elbow extension. Several pharmacologic agents have been used
In some cases, these pseudo-paralyzed muscles percutaneously: anesthetics (lidocaine), diluted
may be present but made ineffective by the spastic alcohol [5], and phenol [6]. As the last is toxic to
antagonist or by elongation caused by a severe surrounding soft tissues and sensory nerve fas-
deformity. Such is often the case for the wrist cicles, it is better to approach the nerve surgically
extensors in severe flexion deformities of the for phenol blocks [7].
wrist. Careful palpation of the muscle belly Whichever substance is used, it may be injected
during attempted active motion may confirm that either around the nerve trunk or in the motor
the muscle is not paralyzed but cannot give endplates of the involved muscles. Nerve trunks are
information of its actual strength. In such cases, fairly easy to locate. Injection at this site diffuses to
an accurate evaluation of individual muscles can other motor nerves, however, with an overlapping
be performed only after spasticity of the antag- response. The motor endplates of individual
onists has been suppressed (see ‘‘Motor blocks’’ muscles are much more difficult to locate and
below). clinicians previously relied on chart drawings [5].
The spastic muscles (flexor, adductor, and This has been facilitated greatly with the use of
pronator muscles) are also usually active, but nerve stimulators. For intrinsic muscles in the
their voluntary movements often are impaired by hand, it is practically impossible to find the motor
co-contractions of other muscle groups. Co- endplates of each individual muscle. One usually
contractions are frequent in the hand, especially chooses to inject the motor branch of the ulnar
of the wrist flexors during attempted finger nerve and, if required, the thenar motor branch.
flexion. They may render clinical examination The effect of a motor block with lidocaine
difficult. In difficult cases, motor blocks and starts 15 minutes after injection and usually lasts
electromyographic studies are helpful. for half an hour. The duration of efficacy is longer
for alcohol and much longer for phenol. Spasticity
yields completely, whereas contracture persists [7].
Fibrous contracture
One then can assess muscle contracture in each
Fibrous contracture involves only spastic
muscle or muscle group. The most frequent
muscles. Unlike spasticity, fibrous contracture is
example in the upper limb is severe spasticity of
permanent and cannot be overcome. It can be
the wrist flexors. Following paralysis, muscles can
alleviated by shortening the involved articular
be tested for contracture and the finger flexors can
segment. For example, posturing the wrist in
be assessed. Injection of a spastic pronator teres
maximal flexion relieves fibrous contracture of the
informs of a possible contracture of the inter-
finger flexors.
osseous membrane.
In cases of severe spasticity, however, clinical
One also can test the nonspastic muscles then,
distinction between contracture and spasticity
now relieved of their spastic antagonists, for
may be extremely difficult to establish. In such
voluntary motor control. In specific cases (eg,
cases, motor blocks may be helpful [4].
extensor carpi radiales muscles) the muscles are
Motor blocks. These blocks require a percutane- present and do contract, but are ineffective
ous injection. It is recommended that they be per- because of progressive lengthening over time,
formed at the very end of the clinical examination secondary to a severe wrist flexion deformity.
so the child remains confident and cooperative Within a spastic group, one can perform
throughout the whole session. a selective block of the predominant spastic
560 C. Leclercq / Hand Clin 19 (2003) 557–564

muscle to evaluate voluntary control and spastic- Primitive reflexes also are sought. They are
ity of the other muscles. For example, after caused by an abnormal sensory motor develop-
injecting the FCU, one can test flexor carpi ment and impair greatly the functional capacity of
radialis (FCR). In the same way, after injecting the limb. One classic example of these primitive
the biceps brachii, one can test the brachialis and reflexes is the asymmetric neck reflex: when the
the brachioradialis. head is turned actively or passively to one side, it
Recently, botulinum toxin has partially sup- produces abduction of the shoulder and extension
planted motor blocks. It produces the same effects of the elbow, wrist, and fingers of the ipsilateral
but with a much easier technique of injection, upper limb, whereas the contralateral limb flexes
a lack of side effects, and a long lasting effect [8]. in all joints.
Injection of botulinum A within the body of Once the motor examination has been com-
a muscle induces a paralysis that starts 10–15 days pleted, an attempt at classification can be made.
after the injection and lasts 3–6 months [9,10]. Zancolli’s classification [12] is probably the most
Another article in this volume is dedicated to popular one:
botulinum toxin and describes its indications in
cerebral palsy (See article by Chin and Graham).  Type I includes the spastic intrinsic-plus
Whether used as a diagnostic tool for preoperative hands, in which spasticity of the interossei
assessment or as part of the therapeutic plan, and lumbrical muscles causes flexion of the
botulinum effectively replaces motor blocks. MP joints and extension of the IP joints,
sometimes associated with a swan neck
Joint range of motion deformity. In this type a wrist flexion de-
Passive range of motion rarely is affected in formity is rare.
isolated cerebral palsy in children. It is impaired  Type II includes the spastic flexion-pronation
more frequently in adults [11] and, when severe, hands with (hyper)flexion of the wrist and
associated neurologic lesions coexist. pronation of the forearm. Three groups are
The precise range of motion of many joints individualized depending on the degree of
may be difficult to assess, not so much because active finger extension. In the first group, with
of spasticity, but because of muscle fibrous the wrist in neutral or near neutral, there is
contractures. full active extension of the fingers. In the
Each individual joint is tested with the in- second group there is nearly complete active
volved muscles fully relaxed. This is performed as extension of the fingers, but only with some
gently as possible so as not to trigger spasticity. If degree of wrist flexion. This group is sub-
fibrous contracture is present, motor blocks do divided further based on the presence (sub-
not assist in assessment of the passive range of group A) or absence (subgroup B) of active
joint motion. Sometimes it is so difficult that wrist extension. In the third group there is no
preoperative examination under anesthesia is active finger extension, even with maximum
necessary to define the range of motion precisely. wrist flexion.
Some joints of the fingers and thumb may have Goldner [4] has produced another classifica-
excessive passive motion, resulting in joint in- tion:
stability. This occurs mainly at the thumb MP
joint (hyperextension and lateral instability) and  In group I the wrist and MP joint can be
at the finger MP and PIP joints (hyperextension). extended at least to neutral. There is active
Finally, a general motor assessment of the grasp and release. The main deficiencies are
upper limb is performed to evaluate global motor delayed speed, slow coordination, and mini-
control. Some standard tests are helpful, such as mal dexterity.
the head-to-knee test, in which the patient is asked  In group II there is weakness of wrist and
to place his hand on his head and then move it to finger extension with mild contracture of the
the contralateral knee. The speed and precision of wrist, finger, and thumb flexors. The thumb
the movement are recorded. These nonspecific remains in the palm during hand extension.
tests involve many of the elements susceptible to The hand is used only as an assist and
perturbation (hypertonia and muscle contracture, a stabilizer.
ataxia, apraxia, and extrapyramidal lesions) and  In group III the wrist and finger flexors are
give information of the potential use of the limb contracted severely. The primary goal of
should a surgical treatment be decided on. surgery is cosmetic improvement.
C. Leclercq / Hand Clin 19 (2003) 557–564 561

 In group IV the hands are spastic and can recognize large figures drawn in the palm,
athetoid. and has a two-point discrimination test of no
greater than 5-10 mm (according to the child’s
House [13] has classified thumb deformities in
age) [14].
four types:
Pain may be present but is difficult to evaluate,
 Type I is an isolated adduction of the first especially in children who may not report it and
metacarpal. may not know how to describe it. It may be linked
 Type II is adduction of the first metacarpal to severe contractures or to a deformed joint.
with flexion of the MP joint. Other causes of pain should be considered. For
 Type III is adduction of the first metacarpal example, the author has treated a young adult
with MP joint hyperextension. with CP with wrist pain caused by Kienböck
 Type IV is adduction of the first metacarpal disease, perhaps related to the severe flexion
with flexion of MP and IP joints. deformity of his wrist [15].
Aside from the thumb classification of House, Functional examination
which is purely descriptive, the author does not
find any of the available classifications particularly This part of the examination tests the actual
helpful, as the clinical picture always varies from use of the upper limb. It is done with standard
one child to another, depending on the amount reproducible tests using a pre-established scenario
and extent of spasticity and paralysis. There are no and simple objects.
two identical cases and often they do not fit It should be video recorded. This usually
accurately in any of the described categories. proves to be a great assessment tool, as it can be
visualized as many times as necessary without
Sensory examination necessitating the child’s presence and cooperation.
The same tests and video recording are repeated
Sensory examination requires, besides the after surgery and serve as a comparison for
child’s cooperation, a certain level of intellectual evaluation of the results of surgery.
capacity and language ability. It is practically
impossible in the small child; it becomes feasible Grasping tests
at age 4–5 years, and more complicated tests such Handing objects to the child tests prehension.
as two-point discrimination become reliable only Thumb-finger pinch often is limited to a lateral
at age 6–7 years. (key) pinch because of the lack of fine voluntary
The basic sensory functions (light touch, pain, control and because of the adducted posture of
temperature) are essentially intact in cerebral the thumb. Grasp generally is preserved, although
palsy, whereas complex sensations (epicritic sen- not always functional because of finger flexor
sibility, proprioception, gnosis) are affected more contractures, extensor weakness, and wrist flexor
severely. co-contractions. Release often proves difficult
Light touch is explored with a smooth point or because of weakness of active finger extension.
a finger, pain with a needle, and temperature with The pick up and release test is done with
tubes of hot (40 C) and cold (melting ice) water. several objects of different size and shape dis-
Epicritic sensibility is explored with discrimina- played on a table in front of the child, asking him
tory tests such as two-point discrimination. or her to pick up each of them and displace it to
Proprioception is tested by vibration (tuning a different spot. This test evaluates not only the
fork) and by the sense of position of the limb: the prehensile capacity of the hand but also the
patient is blindfolded, has the unaffected limb contribution of the whole limb to that function.
placed in one position and is asked to reproduce Quantitative measurements can be made if one
the position with the affected limb. Proprioception introduces the time factor.
is usually more disturbed in the distal part of the Many different tests have been described for
limb. this assessment. In the ‘‘Box and Block Test,’’ the
Gnosis is most affected. Placing an object in patient moves as many wooden blocks as possible
the child’s hand and asking him or her to identify from one compartment to another in 1 minute
it tests stereognosis. Graphesthesia is tested by [16]. Enjalbert’s test [17], designed for stroke
drawing figures or forms in the patient’s palm. patients, involves grabbing a pen presented 40 cm
Sensation is considered satisfactory when the away from the involved upper limb and handing it
child identifies at least three out of five objects, back to the examiner.
562 C. Leclercq / Hand Clin 19 (2003) 557–564

Instrumented objects have been used more General preoperative examination


recently in an attempt to quantify hand grasp.
The aim of this general examination is to
Computerized systems of movement analysis now
evaluate the real benefit the child will get from
allow analysis of the different sequences of motor
surgery, taking into account other neurologic
events and captors integrated in gloves can
impairments, the patient’s age, intelligence, moti-
measure the forces generated by grasp [18,19].
vation, and environment.
Bimanual activities
These give accurate information of the spastic Other neurologic impairments
upper limb’s actual functional ability. Several tests As these children are hemiplegic, the lower
can be used, such as carrying a container with two limb deficit also must be assessed. It is especially
handles, holding one object into which another important to know about the child’s walking
one should be placed (eg, envelope and letter, nest ability and the possible need for walking aids (eg,
of dolls) or holding a ruler while drawing a line crutch, wheelchair). If operations are necessary
with the other hand. for improvement of the lower limb, they usually
are planned before upper extremity surgery.
Questionnaire Associated extrapyramidal signs also should be
The child and family are asked to describe detected. These include the following:
precisely how the hand is used in activities of daily
 Athetosis, which consists of unexpected,
living, such as dressing, self-care, and eating. This
nonvoluntary movements causing a slow os-
can be done in the form of a questionnaire that
cillation of the limbs. It is reduced at rest,
must be adapted to the child’s age. The question-
abolished at sleep, and increased by noise,
naire can be completed during the session or given
fatigue, and emotions.
to the child and family to be completed at home
 Chorea consists of brisk, rapid, and anarchic
(self-questionnaire). In many cases the upper
nonvoluntary movements of variable ampli-
extremity is neglected by the child despite
tude that can involve all territories. In the
functional capacity. In these cases it is possible,
upper limb these contortions of the forearm,
if not probable, that the child persists in ignoring
hand, and fingers make activities of daily
the limb even if functional ability can be improved
living impossible.
by surgery.
 Parkinson syndrome is characterized by the
There are several validated nonspecific ques-
classic triad: resting tremor, plastic hyperto-
tionnaires evaluating hand function (Michigan
nia (predominant in the proximal muscles)
Hand Outcome Questionnaire [20]. To the best of
with the cogwheel sign, and akinesia.
the author’s knowledge, however, there is no
validated questionnaire adapted to the cerebral If these extrapyramidal signs are predominant,
palsied child. they preclude surgical treatment, because the child
is unable to use the hand because of these
Tests nonvoluntary movements. When they are mild,
Many tests have been designed to assess the surgery may be performed when indicated.
functional value of the upper limb. Among the The capacity of the child to communicate must
nonspecific tests, only those testing the simplest be evaluated, seeking visual, hearing, and lan-
functions can be used in patients with CP guage deficits. Behavioral problems, such as
(Frenchay Arm Test, with only five items [21], irritability, inability to concentrate, and emotional
Jebsen test, with seven items [22]). instability, also may constitute contraindications
There are few tests specifically designed for the to surgical treatment if they predominate. In-
spastic upper limb [4]. The ‘‘400 points’’ test [23], telligence is evaluated through the intelligence
which analyzes mobility, grip strength, pick up quotient (IQ). It usually is stated that rehabilita-
and release, and bimanual function, can be used in tion surgery is not indicated when the IQ is lower
children starting from 7 years of age. In children than 70, but this is not absolute. Surgical
younger than 7 years, Rumeau et al [24] use procedures, aimed at improving comfort, cosme-
a bimanual test adapted to each age group. sis, and personal hygiene, still are indicated [25].
The author’s group has chosen to use that
described by Hoffer [14], which tests dressing, Age
personal hygiene, feeding, bimanual activities, Because the neurologic deficit in cerebral palsy
grasp and release, and lateral pinch. is not progressive, early surgery can be planned.
C. Leclercq / Hand Clin 19 (2003) 557–564 563

Sometimes it is necessary to operate very early determining the most appropriate muscles for
because of an increasing deformity. In most cases, transfer.
however, one can wait until the child is old
enough that motor and sensory capacities can be
evaluated accurately and he or she can cooperate Radiography
with surgery and the postoperative rehabilitation. Radiographs are part of the preoperative
Sequelae of cerebral palsy in adults should be evaluation. They are aimed at assessing any
evaluated cautiously before surgical planning, growth disturbance and joint deformity linked to
because usually the patients have adapted already, the spasticity. Satisfactory assessment, however,
functionally and socially, to the handicap, and may not be easy when there is a severe deformity
surgery may be more disturbing than beneficial such as wrist hyperflexion. Contralateral views in
[26]. the same position may be helpful then. In such
Motivation and environment cases, standard radiographs may reveal growth
Evaluation of motivation should take into disturbances of the distal radius and ulna and the
account the patient’s ability to understand the carpus. They also may reveal avascularity of the
modalities and benefits of the proposed treatment lunate (Kienböck disease) [15]. In the elbow there
and to participate actively in the postoperative may be a rare dislocation of the radial head
regimen. Understanding and motivation on the [29,30].
part of the parents are also mandatory. Environ-
mental factors during the surgical period are also
important, such as a rehabilitation center with an Summary
integrated school system and physiotherapists Clinical examination of the upper limb of
experienced in the management of children with a spastic patient is a lengthy and somewhat
CP. All are necessary components of the pre- complicated procedure that is best performed
operative evaluation. together with all the practitioners involved in the
According to Tonkin [27], ‘‘the ideal candidate child’s care. It should be repeated before any
is a cooperative 6-year-old child, with stable decision is made regarding treatment.
family support, who has a predominantly spastic Standard procedures in surgery of the upper
upper limb deformity, with satisfactory hand limb in cerebral palsy are not appropriate, as the
sensibility, hemiplegic or monoplegic and without clinical picture is different for each individual
significant neurological deficits.’’ patient. Surgery should be aimed at correcting
spasticity, muscle/joint contracture, and motor
deficit as required. All these elements there-
Electromyography fore must have been assessed carefully before
EMG studies are often helpful. Static and treatment.
dynamic studies are necessary, but they require Finally, clinical examination detects all local
cooperation on the child’s part [28]. This may be and general contraindications to surgery and
difficult to achieve in children younger than 5 selects the appropriate candidates.
years of age.
In spastic muscles, EMG studies give informa-
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[16] Mathiowetz V, Volland G, Kashman N, Weber K. [27] Tonkin M. The upper limb in cerebral palsy.
Adult norms for the Box and Block Test of manual Current Orthop 1995;9:149–55.
dexterity. Am J Occup Ther 1985;39(6):386–91. [28] Hoffer MM, Perry J, Melkonian GJ. Dynamic
[17] Enjalbert M, Pelissier J, Blin D, Codine P, Lopez electromyography and decision-making for surgery
FM, Simon L. Classification fonctionnelle de la in the upper extremity of patients with cerebral
préhension chez l’hémiplégique adulte. In: Pelissier palsy. J Hand Surg 1979;4(5):424–31.
J, editor. Hémiplégie vasculaire de l’adulte et [29] Pletcher DF, Hoffer MM, Koffman DM. Non-
médecine de rééducation (Problémes en Médecine traumatic dislocation of the radial head in cerebral
de Rééducation. Vol 11). Paris: Masson; 1988. palsy. J Bone Joint Surg 1976;58A(1):104–5.
p. 212–23. [30] Sneineh AKA, Gabos PG, Miller FMD. Radial
[18] Martinet M, Andre A, Hean CC, et al. Capteur de head dislocation in children with cerebral palsy.
contrainte tridimensionel pour l’exploration fonc- J Pediatr Orthop 2003;23(2):155–8.
Hand Clin 19 (2003) 565–571

Functional assessment aided by motion


laboratory studies
Ann E. Van Heest, MD*
Department of Orthopedic Surgery, University of Minnesota, 2450 Riverside Avenue South R200,
Minneapolis, MN 55454, USA
Hand Surgery Section, Gillette Children’s Specialtycare Hospital, Shriner’s Hospital–Twin Cities Unit,
Minneapolis, MN 55414, USA

Cerebral palsy is a primary central nervous extremities; the purpose of the evaluation is to
system (CNS) dysfunction that can lead to confirm a diagnosis of cerebral palsy.
significant functional impairment because of its In most other scenarios, the purpose of the
secondary peripheral manifestations in the upper hand surgeon consultation is for management of
extremity. Depending on the size and location of upper extremity deformity after the diagnosis of
the central nervous system insult, the peripheral cerebral palsy has been made. The hand surgeon
manifestations can vary and need individual must continue to work with the other members of
assessment. This article deals with the assessment the patient’s care team (eg, rehabilitation physi-
protocol necessary for formulation of appropriate cians, neurologists, neurosurgeons, orthopedic
treatment plans. surgeons managing the lower extremity care, and
In summation of the evaluative process, the physical and occupational therapists) to maintain
physician needs to assess mentation, motivation, a multispecialty approach that coordinates ser-
sensibility testing, and static and dynamic de- vices for the patient appropriately. Associated
formities with their resultant functional impair- issues can include mental retardation, seizures,
ments, to synthesize an overall treatment plan, speech disorders, lower extremity involvement
taking into account the child’s capabilities, dis- that affects mobility issues, and timing/coordina-
abilities, and potential in the context of the child’s tion with lower extremity surgeries.
age. Discussion with the parents and the child The hand surgeon most commonly is asked to
is imperative in formulating the individualized evaluate the patient for appropriate treatment
treatment plan and its expected outcome. interventions to improve the child’s use and
appearance of the arm. Initial evaluation may be
Purpose of evaluation more comprehensive to include all aspects of the
evaluation (as described later) or may be more
If a child first presents to the hand surgeon with limited in the very young child or the child with
complaints of delayed development of normal very limited function. For example, a noncommu-
pinch and grasp function and does not yet carry nicative quadriplegic spastic teenager with totally
a diagnosis of cerebral palsy, this occurs most dependent care would have a more limited ex-
commonly at approximately 1–2 years of age. In amination consisting mostly of history with the
this scenario, a complete neurologic evaluation is caregivers and physical examination documenting
necessary, including evaluation of their lower passive range of motion only (if little active motion
exists). In a highly functioning spastic hemiplegic
9-year-old patient with wrist flexion deformity,
* Department of Orthopedic Surgery, University of however, a fairly comprehensive examination
Minnesota, 2450 Riverside Avenue South R200, Min- should be performed to evaluate whether the pa-
neapolis, MN 55454, USA. tient would be a good candidate for surgical in-
E-mail address: vanhe003@tc.umn.edu tervention and for surgical management planning.
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00058-1
566 A.E. Van Heest / Hand Clin 19 (2003) 565–571

The purpose of subsequent yearly or biyearly be interviewed regarding use of the affected limb.
monitoring visits with the hand surgeon is to assess Most commonly children with spastic hemiplegia
for development of contractures and overall de- show premature hand dominance favoring the
velopmental progress regarding age-appropriate unaffected side even as young as 6 months of age;
use of the arm. For example, infants are assessed this may be the presenting complaint leading to
for their ability to bear weight on the arm for sitting the diagnosis. Generalized patterns of upper
and crawling; young children are assessed for extremity use for activities of daily living, com-
bimanual gross motor skills such as dressing and mensurate with the child’s age, are discussed
ball catching; school age children are assessed for with the parents and child, specifically inquir-
fine motor skills such as buttoning and shoe tying; ing about necessary bimanual skills such as cut-
all children are assessed for development of ting meat, tying shoes, zippering, buttoning, and
contractures with growth. If failure to meet de- sports. Often parents complain that the child
velopmental milestones is encountered, a specific never uses the hand at all, but this needs to be
therapy protocol is instituted to help keep the child verified by observation and assessment.
as close to ‘‘on target’’ as their physical disabilities
allow. Therapists are in a unique position to
Physical examination
develop a relationship with the family and child
in patient education regarding use patterns and Physical examination begins with evaluation for
adaptive patterns; therapists are a key source of static deformity by examining the limb for passive
information as to whether the child is making range of motion of the shoulder, elbow, forearm,
appropriate developmental progress. wrist, and hand, evaluating for joint contractures
and shortening of the muscle-tendon units. Passive
General classification range of motion needs to be done slowly to
overcome muscle tone. If there is full passive
Cerebral palsy commonly is classified by topo- mobility of the joints and muscle, no contracture
graphic involvement. Topographic involvement exists. If there is loss of passive range of motion,
means the number of limbs involved: monoplegia then a joint or muscle contractures exists. Most
(one limb), hemiplegia (one arm, one leg), diplegia commonly muscle and joint contractures coexist as
(both legs), triplegia (three limbs), quadriplegia shown in Fig. 1, but may occur separately as shown
(both arms and both legs), and total body in- in Fig. 2. The finger flexor muscles are biarticular
volvement (quadriplegia with mental retardation). muscles, meaning they cross over more than one
The type of muscle tone disorder clinically joint (the wrist joint and the finger joints).
manifested further classifies the disorder; mani- Positioning of the wrist joint in flexion thus allows
festations of central nervous system dysfunction full finger extension if there is no finger joint
can include spasticity, dystonia (athetosis), flac- contractures; however, positioning of the wrist
cidity, or mixed. The amount of central nervous joint in extension does not allow full finger
system control of the peripheral muscles is vari- extension if there is finger flexor muscle contrac-
able and needs to be assessed specifically as part of ture. This is analogous to the intrinsic tightness test.
the patient evaluation. Active range of motion is assessed next,
including specific muscle testing for voluntary
Patient evaluation motor control of antagonist muscles. This is
particularly important for muscles that are
History
considered for tendon transfer, such as the pro-
Because cerebral palsy is associated with low nator teres (for pronator teres rerouting), the
birth weight and prematurity, associated medical flexor carpi ulnaris, extensor carpi ulnaris, or the
problems should be noted, particularly seizures brachioradialis (for wrist extension), the extensor
and mental retardation, as indicators of more pollicis longus (EPL) (for EPL rerouting), and the
global CNS involvement. Developmental motor extensor pollicis brevis and abductor pollicis
delays should be assessed. Past history should longus for control of antagonists to the thumb-
include birth history, medical problems, past in-palm deformity. In some cases the spasticity in
surgical history, medications, and social history, the agonist muscles (eg, pronator teres, flexor
including the child’s educational situation. carpi ulnaris, adductor pollicis) is significant; in
Functional use of the limb needs to be assessed these cases strength or control of the antagonist
next. The parent and child (if old enough) should can be difficult to assess. In these instances,
A.E. Van Heest / Hand Clin 19 (2003) 565–571 567

Fig. 1. (A) Wrist joint contracture and finger/thumb flexors (muscle) contractures coexist in this patient. (B) With wrist
extension, the finger flexors and thumb flexor tighten. Passive finger extension with concomitant wrist extension does not
exist, indicating myostatic contracture of the finger/thumb flexors.

further assessment of antagonist control can be assessed as: does not use, passive assist (poor,
assessed after botulinum toxin injection into the fair, good), active assist (poor, fair, good), and
spastic agonist or by motion laboratory analysis. spontaneous use (partial, complete). Direct ob-
Dynamic deformity is assessed during active servation of the child during bimanual activities
range of motion activities. The dynamic position- and use of videotape review of bimanual skills
ing of the shoulder, elbow, forearm, wrist, fingers, help with appropriate functional use assessment.
and thumb is noted, particularly for grasp, Videotaping allows assessment of the spasticity
release, and pinch function. Age-appropriate tasks encountered during routine ADLs and bimanual
or toys that require two-handed use are helpful in skills; further, it eliminates the stress of perfor-
this assessment. Identification of the specific mance on demand in the physician’s office.
spastic muscle can be determined by the joint Classification of the Functional Use of the upper
position; for example, excessive dynamic wrist extremity provides a baseline that can be used
flexion with ulnar deviation identifies the flexor to help the physician communicate the func-
carpi ulnaris as the spastic deforming force. tional goals of the surgery with the parents.
Palpation of specific spastic muscles also localizes The functional use can be reassessed postopera-
the source of the dynamic imbalance. tively using this scale to assess for functional
improvement.
Functional testing
In the higher functioning child, the Pediatric
The child’s functional use of the hand can be Jebsen Taylor [2] standardized test can be used as
quantified using House’s [1] Classification of a baseline from which to measure the effect of sub-
Upper Extremity Functional Use (Table 1). In sequent treatment and also as a screen of which
this nine-level classification, functional use is subtest functions have the greatest impairment

Fig. 2. (A) Wrist joint contracture with full finger mobility, ie, no myostatic contractures of finger flexors. (B) With
maximum wrist extension there is full digital mobility.
568 A.E. Van Heest / Hand Clin 19 (2003) 565–571

Table 1
House’s classification of upper extremity functional use
Class Designation Activity level
0 Does not use Does not use
1 Poor passive assist Uses as stabilizing weight only
2 Fair passive assist Can hold an object placed in hand
3 Good passive assist Can hold onto object placed in hand and stabilize it for use by other hand
4 Poor active assist Can actively grasp object and hold it weakly
5 Fair active assist Can actively grasp object and stabilize it well
6 Good active assist Can actively grasp object and then manipulate it against other hand
7 Partial spontaneous use Can perform bimanual skills easily and occasionally uses the hand spontaneously
8 Full spontaneous use Uses hand completely independently without reference to the other hand

(eg, pinching small objects versus grasping large tendon transfers in 17 patients with cerebral palsy.
cans). Identification of the specific spastic muscle Only muscles with isolated activity in grasp were
can be determined by the joint position; for used to transfer in hands with weak grasp; only
example, excessive dynamic thumb adduction muscles with isolated activity in release were used
without other thumb deformity identifies the to transfer in hands with poor release. The two
adductor pollicis as the spastic deforming force. most common procedures were FCU to EDC
transfer (nine procedures) and FCU to extensor
carpi radialis brevis (ECRB) (three procedures).
Motion laboratory assessment
This approach assumes that no change in phasic
Dynamic EMG testing is another diagnostic activity occurs. These investigators assumed that
tool that has been used to help assess motor tone all muscles in cerebral palsy are phase-dependent;
and phasic control of specific muscles [3,4]. In only when the FCU fired in release was it
major centers, motion laboratories for gait transferred to the EDC or ECRB for release.
analysis have been used extensively for lower Mowery et al [9] evaluated 16 transferred
extremity tendon transfer surgery [5–7], and the tendons in eight patients with pre- and post-
same technology can be used for upper extremity operative dynamic EMG testing. Four of the 16
assessment. The specific role of the use of the muscles showed changes in their electrical activity
motion laboratory in the upper extremity, how- postoperatively: three muscles that fired continu-
ever, continues to be under investigation as ously became phasic and one muscle changed
a promising tool for more objective assessment phase to match its recipient. Phasic versus con-
of muscle control for planning appropriate tendon tinuous spastic activation can be assessed, as can
transfer surgery [3,4,8–11]. determination of central control of muscles as
One use of the motion laboratory has been for children are observed carrying out functional
assessment of correction of elbow flexion de- tasks. This study brings forth the concept that
formity. Kozin et al [12] have shown that biceps or muscles may be able to change phase with trans-
brachialis spasticity can lead to elbow flexion fer, even in cerebral palsy.
deformity; preoperative dynamic electromyogra- In the author’s laboratory, we have used the
phy (EMG) can assess spastic tone and phase motion laboratory for planning tendon transfers
control of each muscle to direct treatment to the for forearm rotation and wrist extension, as
specific offending muscles. shown in Fig. 3. The two video frames show
Hoffer et al [8] has focused their use of a front and side view of the child performing the
dynamic EMGs to assess differences in phasic Pediatric Jebsen hand test. Looking at the two
control of muscles. Hoffer described using dy- angles simultaneously allows assessment of the
namic EMG assessment to characterize preoper- deformity in the sagittal and coronal planes. For
atively the extensor digitorum communis (EDC), example, this child can be seen to demonstrate
the flexor carpi ulnaris (FCU), the flexor digito- a wrist flexion and ulnar deviation deformity. The
rum superficialis (FDS), the flexor digitorum EMG data on the right show 3 seconds of
profundus (FDP), the extensor carpi radialis and electrical-myographic activity of four muscles:
brachioradialis, and the pronator quadratus the biceps, the pronator teres, the flexor carpi
muscles. Each muscle was described as firing in ulnaris, and the extensor carpi radialis longus/
grasp or in release. He described the results of brevis. Needle electrodes are used for the pronator
A.E. Van Heest / Hand Clin 19 (2003) 565–571 569

Fig. 3. Motion laboratory assessment with dynamic Fig. 4. Motion laboratory assessment with dynamic
EMG. The two video frames on the left show a front and EMG. This motion laboratory assessment demonstrates
side view of the child performing the Pediatric Jebsen spastic continuous activity of the flexor carpi ulnaris, yet
hand test. Looking at the two angles simultaneously there is phasic control of wrist extension. The flexor
allows assessment of the deformity in the sagittal and carpi ulnaris fires continuously during grasp and release,
coronal planes. For example, this child can be seen to indicating significant spasticity that masks this patient’s
demonstrate a wrist flexion and ulnar deviation de- ability to extend the wrist actively. Electrical activity in
formity. The EMG data on the right shows 3 seconds of the extensor carpi radialis brevis and longus seems to be
electrical myographic activity of four muscles: the biceps, firing appropriately during grasp and relaxing during
the pronator teres, the flexor carpi ulnaris, and the release, but is not seen clinically as it is overpowered by
extensor carpi radialis longus/brevis. Needle electrodes the flexor carpi ulnaris spasticity.
are used for the pronator teres and the flexor carpi
ulnaris. Surface electrodes are used for the biceps and the In Figs. 3 and 4, the two patterns of wrist
radial wrist extensor muscles. The box encompassing the flexion deformity are demonstrated. In Fig. 3, the
central one third of the EMG data highlights the one flexor carpi ulnaris is seen to fire phasically, ie, it
second of activity that is shown simultaneously on the
turns off and on according to the function
video frames on the left. This motion laboratory study
performed and the position of the limb. In Fig.
demonstrates continuous firing of the flexor carpi
ulnaris. Lack of normal firing during grasp and re- 4, the flexor carpi ulnaris does not show selective
laxation during release indicates lack of phasic activity control, but rather fires continuously in a spastic
and poor cortical control of this muscle. pattern. This can be appreciated best by watching
the continuous video with simultaneous EMG
over time and through activities.
teres and the flexor carpi ulnaris. Surface elec-
Decision making for tendon transfer surgery is
trodes are used for the biceps and the radial wrist
based then on the pattern of muscle spasticity and
extensor muscles. The box encompassing the
central one third of the EMG data highlights the
one second of activity that is shown simulta-
neously on the video frames. This motion analysis
study allows for assessment of the spasticity pat-
terns of the muscles and helps determine whether
the child is able to control actively the muscles for
functional use.
The motion laboratory has shown several
patterns of EMG activity in the muscles tested. In
Fig. 5, the wrist extensors show good phasic
control. The patient is able to fire the wrist
extensors actively. The FCU and FCR leads show
continuous spastic activity with no phasic control.
In such a pattern (wrist extensors in phasic control Fig. 5. Motion laboratory assessment with dynamic
and wrist flexors in spastic activity), the patient is EMG. This motion laboratory assessment demonstrates
treated with a flexor pronator slide. The patient was phasic control of flexor carpi ulnaris. Appropriate firing
skeletally mature, and with reduction of the flexion during grasp and relaxation during release is seen cor-
forces, the extensor control could be unmasked. responding to functional grasp and release of the cans.
570 A.E. Van Heest / Hand Clin 19 (2003) 565–571

voluntary control. For muscles with good volun-


Box 1. Objects for assessment
tary control, tendon transfer is recommended. In
of stereognosis function
Fig. 3, the flexor carpi ulnaris has phasic control
and would be transferred to the extensor carpi 12 common objects
radialis brevis. In Fig. 4 the FCU has uncon- Pill
trolled spasticity and would be lengthened with Penny
use of another muscle for transfer, such as Button
brachioradialis or extensor carpi ulnaris. In Fig. Key
5, a flexor pronator slide was done to decrease Paper clip
flexion forces and unmask a phasic, well con- Safety pin
trolled wrist extensor. Spoon
Cube
Sensibility Marble
Sensation can be evaluated by stereognosis, Rubber band
two-point discrimination, and proprioception. In String
the author’s review of 40 children with spastic Pencil
hemiplegia [13], the author has found that stereog-
nosis is the most sensitive discriminator of degree of The first six objects require fine discrimination
sensibility impairment as shown in Table 2. In this between similar sized small objects, and the
study, 97% of children with spastic hemiplegia had second six objects require gross discrimination
a stereognosis impairment using the 12 objects between larger objects and different materials.
shown in Box 1. The first six objects discriminate
fine motor function and the second six objects
discriminate gross motor function. All children
demonstrated 12/12 object recognition on the Summary
unaffected side, verifying understanding of the test.
Furthermore, this study found that those children The physician needs to integrate the results of
with severe sensibility impairment had a significant the assessment of overall patterns of functional
size discrepancy when compared with the un- use (Table 1), static contractures, dynamic defor-
affected side. The shortened limb can be a useful mities of motor imbalance for multiple levels of
clue to underlying sensibility deficiency, particu- involvement (shoulder, elbow, wrist, and hand),
larly in the child too young or too retarded to and sensory deficiencies. This information is
perform a sensibility assessment reliably. Children combined with a general assessment of the child’s
with sensibility deficiencies need to be coached to mentation, motivation, and generalized medical
use the eyes rather than their touch for afferent condition.
feedback. Several studies have indicated that poor Certain patients benefit most from surgical
sensation is not a contraindication for surgery intervention in cerebral palsy. Patients with
[14,15]. In fact, one study has reported an spastic deformities or flaccid control of specific
improvement in sensibility function after surgical movements can be helped significantly. In patients
intervention [14], presumably associated with in- with flaccid control of certain movements, such as
creased postoperative functional use. absent ability to extend the wrist or abduct the
thumb, surgery is centered on tendon transfers to
augment the patient’s ability to perform that
Table 2 movement. In patients with excessive spasticity or
Sensibility deficiencies musculotendinous contracture, surgery is centered
Type of sensibility testing Percentage of
on muscle lengthening. In general, patients with
patients impaired athetosis are not treated surgically; the only
Stereognosis 97% surgical treatment considered for the athetotic
2-point discrimination 90% patient is fusion, as this helps preposition the limb
Proprioception 46% in a desired position and ‘‘simplifies’’ the system
From Van Heest AE, House JH, Putnam M. for the patient to control.
Sensibility deficiencies in the hands of children with Sensibility deficiencies do not preclude effective
spastic hemiplegia. J Hand Surg [AM] 1993;18:278–81; tendon transfer but do limit the overall use of the
with permission. limb; patients with sensibility deficiencies need to
A.E. Van Heest / Hand Clin 19 (2003) 565–571 571

be coached to use visual input as their afferent [5] Perry J. The use of gait analysis for surgical
information. recommendations in traumatic brain injury. J Head
Motor deficiencies can be assessed by observa- Trauma Rehabil 1999;14:116–35.
tion, examination, functional testing, and motion [6] Sutherland DH, Santi M, Abel MF. Treatment of
stiff-knee gait in cerebral palsy: a comparison by
laboratory analysis. Combining an assessment of
gait analysis of distal rectus femoris transfer versus
shoulder, elbow, forearm, wrist, thumb, and finger proximal rectus release. J Ped Ortho 1990;10:
abilities and disabilities helps provide the physician 433–41.
with an overall plan of upper limb reconstruction [7] Water RL, Frazier J, Garland DE. Electromyo-
using soft tissue releases, tendon transfers, and joint graphic gait analysis before and after operative
stabilization procedures to address the upper limb treatment for hemiplegic equinus and equinovarus
functional deficiencies. The ideal candidate for deformity. J Bone Joint Surg Am 1982;64:284–8.
tendon transfer surgery is 7 years of age or older so [8] Hoffer MM, Perry J, Melkonian G. Dynamic
they can be cooperative with postoperative re- electromyography and decision-making for surgery
habilitation and motivated to improve the use of in the upper extremity of patients with cerebral
palsy. J Hand Surg [Am] 1979;4:424–31.
their limb. Children with passive use of their limb
[9] Mowery CA, Gelberman RH, Rhoads CE. Upper
(Functional Use Classification levels 1–3) can be extremity tendon transfers in cerebral palsy: elec-
improved most, on average 2.7 levels to active use tromyographic and functional analysis. J Pediatr
of their limb (Functional Use Classification levels Orthop 1985;5:69–72.
4–6) [16]. [10] Pinzur MD. Dynamic electromyography in func-
An overall treatment plan is synthesized, tak- tional surgery for upper limb spasticity. Clin
ing into account the child’s capabilities, disabil- Orthop Rel Res 1993;288:118–21.
ities, and potential, in the context of the child’s [11] Thepaut-Mathieu C, Maton B. The flexor function
age and expectations. The assessment techniques of the m. pronator teres in man: a quantitative
discussed in this article are the first step to ap- electromyographic study. Eur J Appl Physiol 1985;
54:116–21.
propriate treatment.
[12] Kozin SH, Keenan MH. Using dynamic electromy-
ography to guide surgical treatment of the spastic
References upper extremity in the brain-injured patient. Clin
Orthop Rel Res 1993;288:109–17.
[1] House JH, Gwathmey F, Fidler M. A dynamic ap- [13] Van Heest AE, House JH, Putnam M. Sensibility
proach to the thumb-in-palm deformity in cerebral deficiencies in the hands of children with spastic
palsy. J Bone Joint Surg [Am] 1981;63:216–25. hemiplegia. J Hand Surg [Am] 1993;18:278–81.
[2] Taylor N, Sand PL, Jebsen RH. Evaluation of hand [14] Dahlin LB, Komoto-Tufvesson Y, Salgeback S.
function in children. Arch Phys Med Rehabil Surgery of the spastic hand in cerebral palsy.
1973;54:129–35. Improvement in stereognosis and hand function
[3] Hoffer MM. The use of the pathokinesiology after surgery. J Hand Surg [Br] 1998;23:334–9.
laboratory to select muscles for tendon transfers in [15] Van Heest AE, House JH, Eliasson AC. Hand
the cerebral palsy hand. Clin Orthop Rel Res function in children with cerebral palsy after upper-
1993;288:135–8. limb tendon transfer and muscle release. Dev Med
[4] Hoffer MM, Perry J, Melkonian G. Postoperative Child Neurol 1998;40:612–21.
electromyographic function of tendon transfers in [16] Van Heest AE, House JH. Upper extremity surgical
patients with cerebral palsy. Dev Med Child Neurol treatment of cerebral palsy. J Hand Surg [Am]
1990;32:789–91. 1999;24:323–30.
Hand Clin 19 (2003) 573–584

Casting, splinting, and physical and occupational


therapy of hand deformity and dysfunction
in cerebral palsy
Judith Wilton, MS, GradDipHthSc, BAppSC, OT
Hand Rehabilitation Specialists, 10 Altona Street, West Perth, WA 6005, Australia

Significant occupational and physical therapy For children with more severe motor dysfunc-
time and resources are directed to the manage- tion in whom head and trunk control are affected
ment of the upper limb in cerebral palsy (CP). The by spasticity and persistent postural reflexes,
evidence to support therapy interventions for the postural control for effective upper limb function
upper limb is not strong [1]. This in part reflects is achieved only through adaptive seating. As
the difficulties of research [2] rather than a lack of children get older, the requirements for schooling
critical review of practice. Experience and demand longer periods of time seated. Therapists
logic underpin many therapeutic applications. thus should consider how restrictions imposed by
This presentation details the available options, the chair and table in upper limb movement may
the rationale for their use, and the results of potentiate deformity and what strategies are
treatment. needed to address it.
Altered sensibility commonly is identified as
a contributing factor to impaired hand function in
Prerequisites for hand function children with CP. Much of the literature has
Efficient performance of the upper limb focused on the measurement of sensory deficits,
depends on proximal control and dynamic stabil- with two-point discrimination and stereognosis
ity of the trunk and shoulder girdle. From a stable identified as the most common tests [7–9]. Recent
base, distal mobility of the limb enables partici- studies of children with hemiplegia have estab-
pation in age-appropriate occupational tasks. lished a strong relationship between tactile sensi-
Improvement in scapulohumeral and trunk con- bility and dexterity [10] and fingertip force
trol is a major focus of neurodevelopmental regulation during object manipulation [9,11].
therapy (NDT), one of the most commonly used The relationship between sensibility and hemi-
approaches for treatment of children with CP. plegic hand performance in functional bimanual
NDT techniques attempt to alter muscle tone activities, however, was not as strong [10]. The
during movement to facilitate normal movement potential to improve hand sensation by systematic
patterns and postural reactions under the premise sensory education programs, shown to be effective
that improved postural control improves func- following stroke [12–14], has yet to be determined
tional skills [3]. Investigations into this premise in in children with CP.
relation to the upper limb determined the effects
of NDT on quality of upper limb movements [4] Principles of intervention options
and reaching [5,6]. Although improvements were
evident, they were not greater than reach training Therapeutic modalities specific to hand func-
using principles of motor learning or occupational tion essentially fall into two categories—those
therapy directed toward functional skills. that have an impact on hypertonicity and
associated contracture and those that facilitate
functional use of the hand related to active motion
E-mail address: turnwil@ozemail.com.au and dexterity. In the absence of extensive evidence
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00044-1
574 J. Wilton / Hand Clin 19 (2003) 573–584

under-pinning the therapeutic modalities as ap- sient lengthening depending on the viscoelastic
plied to typical patterns of deformity of the hand properties of the tissues. This elongation reverses
and wrist. once the force is relaxed. This elastic response is
associated with unfolding of tissue and temporary
realignment of collagen fibers within the connec-
tive tissues. The resolution of contracture through
Hypertonicity and contracture
the application of low-load prolonged stress to the
When addressing the increased muscle tone in contracted tissues at the end of their available
children with CP, it is useful to distinguish range ultimately depends on the ability of the cells
between the neural or reflexive components—the to sense and transduce the mechanical force into
tonic muscle contractions—from the non-neural biologic action and to grow [20,23,27].
or mechanical components, the viscoelastic prop- The response of shortened muscles to stretch,
erties of muscle and connective tissue associated using plaster cast immobilization, has been
with contraction and stretch [15,16]. Spasticity, explored in numerous animal studies. Adult
a somewhat imprecise term in clinical practice, muscle responds to stretching by adding new
refers to the neural component of hypertonicity, sarcomeres in series, thereby returning the sarco-
that is, the velocity-dependent increase in stretch meres to their optimum tension-generating length
reflex activity [17]. The altered mechanical prop- with no change in tendon length. In growing
erties of the muscle and associated tissues occur in muscles, however, the initial increase in number of
response to the abnormal conditions imposed on sarcomeres up to day five is followed by a decrease
the muscle by the spasticity. In turn, muscle in sarcomere number, thereby decreasing muscle
shortening may participate in the generation and fiber length. Muscle tendon length is maintained
maintenance of spasticity [16,18]. by lengthening of the tendon [23,28,29]. These
Strategies that address muscle contracture findings suggest that extended casting protocols
affect the neural and mechanical components of for young children should consider potential to
the hypertonicity present. Studies by Nash, increase tendon length rather than to influence
Neilson, and O’Dwyer [19,20] suggest that neural muscle fiber length. It is stressed also that cast
and non-neural components of hypertonicity lengthening of muscle contracture should be
should be considered as reducing spasticity alone gradual, because decrease in sarcomere number
and not altering muscle contracture. Following is greater than decrease in length of muscle
electromyographic biofeedback training over 10 connective tissue [23]. Potential exists for
weeks, subjects were able to reduce spasticity by muscle fiber breakdown from too fast or forceful
50%, but no associated change in muscle con- stretching.
tracture was measured, nor improvement in Although stretch is essential to muscle growth
voluntary movement evident. To address contrac- and in maintenance of functional length once
ture and hypertonicity effectively, it is recommen- growth has stopped [30], the effectiveness of
ded therapists use modalities that prevent or passive range of motion exercises depends on
reverse contracture in target muscles for sufficient their frequency. Long-term stretch, applied con-
duration each day to promote muscle growth [19]. tinuously for periods greater than 6 hours, has
During growth and in response to changes in been shown to be most effective [31,32]. Casting
posture, the functional length of the muscle is and splinting are best in applying low-load
adjusted by altering the number of sarcomeres in prolonged stretch to contracted tissues. Experi-
series for optimum force generation and power ence suggests that, in muscles with hypertonicity,
output [21]. When movement does not put the shortening of muscle and connective tissues recur
joint through a full range of motion and daily unless stretch is maintained [20,33].
passive range of movement or posturing does not
adequately maintain range, adaptation of the
muscle results in contracture. This adaptation, Splinting and casting
a combination of shortening of muscle fibers and Published studies in the last 50 years that have
remodeling of muscle connective tissue [19,22,23], addressed the issue of hand splinting in the
is accompanied by changes in the skin and presence of spasticity have tended to reflect the
periarticular tissues [24–26]. theoretic basis of therapy at the time [34–40].
When safe forces are applied to tissues Although direct comparison between studies is not
statically or cyclically, they demonstrate a tran- possible, the vast majority identified improvement
J. Wilton / Hand Clin 19 (2003) 573–584 575

in range of motion associated with splint wearing greater grasp skills [57,58]. No significant rela-
[41–43]. tionship was found between splint type and
Static splints and casts maintain the joint in changes in hand function. Functional gains were
one position with the goal of stabilizing it for associated with splint wear. In practice, splints are
efficient transfer of muscular forces to distal designed to meet specific objectives identified by
joints. Serial static splints and casts are designed the patient or their caregiver. In many instances
to lengthen tissues and correct deformity through splints compensate for functional deficits in hand
application of gentle forces sustained for extended grasping or pointing to secure toys, eating
periods of time [44]. Splints are remolded and utensils, and writing and computing devices.
casts replaced at intervals that allowed for tissue When a variety of postures of the upper
response to the lengthened position. Casting has limb are required in the performance of func-
biomechanic and neurophysiologic effects. Bio- tional tasks, rigid correction of deformity is not
mechanic effects relate to changes in the length of always compatible with function. It is probably
muscle and connective tissues reversing the one of the key issues for noncompliance with
histologic changes that occur in tissues main- rigid splinting and the preference for use of
tained in a shortened position. The exact neuro- custom made or commercial neoprene and Lycra
physiologic effects of casting on spasticity are splints. These splints use a wraparound design
undefined. It is proposed that inhibition results with inserts to position the thumb and reinforce-
from decreased sensory input from cutaneous ment achieved by way of splinting material or
and muscle receptors during the period of immo- metal inserts.
bilization. The effects of neutral warmth and Dynamic Lycra splints use the inherent prop-
circumferential contact also are believed to erties of the fabric and design features of the
contribute to modification of spasticity. garment to create a low force to resist the spastic
Much of the research on upper limb casting in muscle action while also facilitating the antagonist
the presence of spasticity is undertaken in a single action. The mechanical properties of dynamic
case study design. Two groups of studies are Lycra arm and hand splints have been established
seen—those using mobilizing principles with in studies involving normal and hemiplegic sub-
a series of circumferential casts worn for 24 hours jects [18,59]. More extensive Lycra body splinting
per day for periods up to 4 weeks [45–50] and in children with CP [60] also showed improved
those in which a single cast is bivalved and worn dynamic upper limb function, with reduction in
for periods of 3–5 hours per day for many months involuntary movement and improved patterns of
[33,44,51,52]. In the first group, serial casts movement associated with a reduction in muscle
applied to elbow and wrist flexion contractures tone. Thirteen of fourteen subjects experienced an
for 24 hours per day over several weeks resulted in immediate reduction in involuntary movement,
significant gains in range of motion. Intermittent with six maintaining some improvement after
static casting of the wrist also demonstrated removal of the body splint. Although preliminary
improvements in range of motion, quality of research suggests Lycra splints have potential to
movements, and functional use of the hand. influence involuntary movement and apply low
Biomechanically, bivalved casts achieve wrist stretching force to the limb in people with CP
immobilization as effectively as a thermoplastic without compromising comfort or functional use
splint. The studies that used static bivalved casts of the limb, further controlled clinical trials are
to immobilize the wrist joint for function for needed.
extended periods provided no explanation re-
garding choice of material. Choice may relate to
Functional strength
the skills of the therapist in splinting or casting,
ease of fabrication, cost, comfort, and aesthetics. Current understanding is that spasticity is not
Splints to facilitate functional use of the hand the primary cause of voluntary movement impair-
include the wrist splints with reflex inhibiting ment [20]. The perception of greater strength in
components [53,41], a neoprene splint to position the hypertonic muscle, with weakness in its
the thumb in abduction and the forearm in antagonist, is in part caused by the techniques
supination [54], and splints to position the thumb used to determine strength. As skeletal muscle is
in abduction [55,56]. Studies that investigated highly adaptable, its structural characteristics are
splints worn by children with CP reveal trends determined by its conditions of use. Shortened
toward more normal movement patterns and muscles may seem strong at normal length
576 J. Wilton / Hand Clin 19 (2003) 573–584

because they are tested in their optimal position. play and peer interaction influenced fine motor
Associated changes in passive tension through skills, improving function. Therapists therefore
shortening of the connective tissue elements also are encouraged to use activities of daily living
may contribute to perceived strength. Weakness (ADL), play, and recreational and vocational
in lengthened muscles is the result of remaining in activities as an integral part of therapy [33,63].
an elongated position beyond the neutral physi-
ologic rest position but not beyond the normal
Analysis and treatment of hand dysfunction
range. Muscles seem weaker because they are not
tested at their optimal length. Constant contrac- The wrist and hand present a complex in-
tion of the muscle when allowed to shorten also teraction of intrinsic and extrinsic musculature in
may exaggerate the rate and quantity of sarco- which hypertonicity dictates the predominant
mere loss, thus weakening the muscle [21]. The pattern of deformity. Performance of an isolated
hypertonic muscle may be constrained during movement may elicit a different pattern from that
voluntary movement by the passive mechanical evident when attempting to use the hand in
properties of the muscle itself. a functional task. Analysis of patterns of function
Lengthening the shortened connective tissue of the wrist and digits during movement and
elements in the muscle, together with modifica- during function provide the basis for treatment
tion of the functional length with adaptation of decisions. In addition to the usual tests of passive
sarcomere number, has potential to modify and active range of motion, information from
‘‘strength’’ of the muscle. Improvement in parents or caregivers about the posture of the limb
strength of wrist extensor musculature described during sleep often can assist in determining
following serial progressive casting [50] is in part whether contracture is present.
the consequence of muscle length adaptation. In 1981, Zancolli and Zancolli [65] described
Following the period of immobilization, therapy a surgical classification of spastic hand deformities
is required to assist weaker muscles to work in the wrist and fingers, whereas House, Gwath-
against gravity and to perform functional grasp. mey, and Fidler [66] identified four patterns of
Splints that offer support to the joint but allow deformity in the thumb. Building on these
active motion are preferable to rigid immobiliza- classifications, the following model was developed
tion. to facilitate analysis of the anatomic and bio-
It is observed that repetitive use of the hand in mechanic components of deformity and dysfunc-
functional tasks has the potential to further tion. Patterns of deformity may evolve over time.
increase strength and endurance. Further investi-
gation is required, however, to validate grip and
Pattern 1: minimal wrist flexion in function,
pinch strength exercise protocols.
thumb adduction
Children with this deformity have mild spas-
Manipulation and dexterity
ticity in flexor carpi ulnaris (FCU), which means
Improvement in manipulation and dexterity that reach to grasp, occurs with slight wrist flexion
are aims for children with CP with milder motor and ulnar deviation. The wrist extensor muscles
difficulties [61,62]. Hand intrinsic muscular con- can extend against the resistance of hypertonicity
trol is essential to achieve fine motor coordina- in FCU and there is no evidence of hypertonia in
tion. Therapy aims to facilitate isolated thumb the finger musculature. In a large number of
and finger stability and mobility, accurate pat- patients, no deficit is seen in the thumb, but when
terns of pinch and grip of objects of varying sizes, present the deformity pattern is adduction at the
shapes, and textures, translation and rotation of carpometacarpal (CMC) joint from a combination
objects within the hand, bilateral manipulation, of contraction and contracture in the adductor
and strength and endurance to meet functional pollicis (AP) and first dorsal interosseous (DI)
demands. muscles. Some CMC joint extension and abduc-
The efficacy of occupational therapy programs tion are restricted but with no limitations in
to address development of fine motor skills in motion of the metacarpophalangeal (MCP) and
preschool children has been established in several interphalangeal (IP) joints.
studies by Case Smith [61,63,64]. Although the Full passive range of motion (ROM) is avail-
number of children in these studies with a di- able at all joints of the wrist and fingers with the
agnosis of CP was small, the therapist’s use of possibility of reduced ROM of the thumb–index
J. Wilton / Hand Clin 19 (2003) 573–584 577

finger web space. Imbalance of muscles acting splint design has been found that resolves this
across the joints can contribute to instability. problem effectively.
Hyperextension seen at the proximal interphalan- People with this pattern of deformity have
geal (PIP) joints results from wrist flexion in- good grasp and release and pinch; however, fine
creasing the distance the extensor digitorum manipulation is impeded by thumb dysfunction.
communis (EDC) tendons traverse before insert- Therapy is focused on activities requiring grasp
ing at the base of the middle phalanx. Great and pinch of objects of a wide variety of sizes and
variability exists in the population as to ‘‘normal’’ shapes to encourage thumb opposition and hand
mobility of these joints, and unless instability manipulation. Placement of objects for manipu-
impedes function it need not be addressed. lation should use a variety of wrist postures, with
vertical play one of the ways to promote wrist
extension.
Therapeutic intervention
Maintenance of full extensibility of tissues is
Pattern 2: moderate wrist flexion in function,
a primary goal. Function and play activities
active wrist extension
that incorporate weightbearing are used to
provide regular stretch to FCU. In the presence People with this deformity use a tenodesis-type
of contracture of the thumb web space, a serial action, approaching objects with significant wrist
splint worn during nonfunctional times ad- flexion with extension of the MCP and PIP joints
dresses the contracture without compromising of the fingers (Fig. 1A). Active flexion of the
function. fingers is associated with extension of the wrist.
Designing functional splints to stabilize the Control of the speed and force of finger flexion is
thumb in a position for opposition is a significant a common problem. The hypertonicity is located
challenge. To control the thumb joints, splint predominantly in flexor digitorum profundus
components must control the thumb metacarpal (FDP) and flexor digitorum superficialis (FDS),
against the forces of spasticity. When the web with mild hypertonicity located in FCU and flexor
space is shortened by skin contracture, the carpi radialis (FCR). Strength in the wrist
difficulty in directing the abducting force to the extensor muscles can overcome the resistance in
metacarpal is increased. Although neoprene or the wrist flexors, but EDC cannot extend the
Lycra are more acceptable splinting materials for fingers with the wrist approaching neutral. EDC is
young children, their elasticity and lack of a critical player in this pattern, as it is compro-
contour require careful design to ensure appro- mised by its incapacity to generate tension when
priate application of forces to the thumb. Com- constantly working in a lengthened position, and
binations of low temperature thermoplastic by its size and overall smaller capacity to generate
materials and Lycra or neoprene may better tension as compared with FDS and FDP [67]. The
achieve this objective, particularly in older chil- thumb metacarpal generally is held in an adducted
dren in whom the tissue forces are greater. It is position by hypertonicity in the AP and first DI
important to ensure that functional thumb splint- muscles. Extensor pollicis longus (EPL) and
ing does not impede use of the thumb; no single extensor pollicis brevis (EPB) act across the

Fig. 1. Pattern 2 with and without dynamic Lycra splint. (A) Approach to grasp is associated with significant wrist in
flexion with hyperextension of the MCP joint. (B) Dynamic Lycra splint facilitates a balance between wrist and finger
musculature when approaching objects for grasp.
578 J. Wilton / Hand Clin 19 (2003) 573–584

MCP joint, creating a hyperextension deformity nities to use newly acquired movement patterns and
on reach, while not impairing thumb flexion. consolidate gains.
No deficits are evident in the passive range of The thumb presents a two-fold challenge.
motion of the wrist. Shortening may be demon- First, the presence of contracture of the thumb–
strated in FDP and FDS, however, with combined index web space, and second, diminished active
finger and wrist extension. Daily stretching pro- motion to position the thumb for effective
tocols in association with weightbearing on the opposition. Resolution of contracture requires
hand with extension of the wrist and fingers may serial progressive splinting, preferably at night so
maintain length without need for splinting or as not to impede performance of functional
casting. Contracture of the index thumb web activities. Although thumb mobility is desirable,
space is generally present. Persistent extension of thumb stability in a position for opposition to the
the MCP in combination with adduction of the fingers is more important. Achieving this stability
CMC joint can lead to instability of the MCP is not easy in a situation in which spastic muscles
joint capsule and ineffective force transmission have good moment arms, and splinting levers are
through the thumb during pinch. small. Splinting materials must have sufficient
In people with this deformity, grasping is strength to resist the force of AP and extrinsic
impaired by reduced thumb web span, limiting extensors to stabilize the CMC and MCP joints.
the area of the palm of the hand available for Correction of the wrist position and thus
object contact. In addition, diminished control of reduction of the distance over which the EDC
wrist extension in conjunction with flexion of the tendons traverse at the wrist resolves much of the
fingers often results in failure to secure the object hyperextension deformity at the MCP and PIP
between the fingers and thumb or the fingers and joints of the fingers. Splinting for the PIP joints is
palm. Successful grip depends on the size and required only if joint instability compromises
shape of objects in relation to the size of the hand. function, particularly for switching devices or
Transverse volar grip is the most effective because computer access. Small splints that restrict PIP
of the tenodesis action in grasping, with effective- joint extension beyond neutral can be fabricated
ness of lateral and two-point pinch dependent on and worn for specific functional tasks, but
the size and shape of the object. compliance with long-term wear is problematic.

Pattern 3: wrist flexion >20 in function,


Therapeutic intervention
no active wrist extension
The objective of intervention is to gain better
coordination between wrist and finger muscular In people with this pattern, moderate spasticity
action. Treatment is designed to combine re- is located in FCU and FCR and the FDP, FDS,
education of movement patterns in functional tasks and palmaris longus. Wrist extensor muscles are
that require wrist extensors to work in their mid weak, constantly working in a position of sig-
range of motion, maximize action of EDC for finger nificant wrist flexion. Mid range flexion of the
extension, and gain control over speed and force of fingers is possible, but active insufficiency in FDS
contraction of finger flexors during grip. Dynamic and FDP impairs strength of grip. Hyperexten-
Lycra splints (Fig. 1B) can assist in achieving this sion of the PIP joints is present (Fig. 2). This
objective, but rigid splints are incompatible with pattern is more common in older children and
functional use of the hand. The Lycra fabric allows adults and reflects the impact of growth and tissue
movement but desires to return to its predeter- shortening over time.
mined resting length. Looking like a glove with The significant feature of this pattern is the
inclusion of fingers to prevent migration, the deficit in extensibility in the wrist flexor and
multiple components across the dorsal and volar extrinsic finger flexor musculature, resulting in
aspects are designed to provide directional pull and loss of extension ROM. Shortening also may be
so facilitate movement. The thumb is included with present in EDC, limiting full passive finger flexion.
addition of small thermoplastic components if The predominant posture of the thumb is
additional stability or positioning is required. adduction at the CMC joint and hyperextension
Initial splint application should be incorporated of the MCP joint as described in the previous
into a therapy program directed toward mastery of classification.
movement patterns required in specific occupa- People with this deformity grasp objects
tional tasks. Repetition in ADL provides opportu- between the finger and thumb pads, as it is not
J. Wilton / Hand Clin 19 (2003) 573–584 579

Fig. 2. Examples of pattern 3 deformities. (A) In association with significant wrist flexion tension on EDC contributes to
hyperextension in the PIP joints, while AP and contracture influence the thumb CMC joint and EPL extends the IP joint
of the thumb. (B) The right hand illustrates the resting position of the wrist reinforced by gravity and wrist and finger
hypertonicity. Greater degrees of extension in the resting position of the left wrist and fingers are the outcome following
18 days of serial casting.

possible to orientate the palm toward the object use as an assist/stabilizer to the dominant hand.
because of significant wrist flexion. For patients in whom bilateral involvement exists,
intervention often is directed at achieving the
hand function requirements for a specific task,
Therapeutic intervention
such as wheelchair control or activating commu-
The objective of intervention is to improve
nication devices (Fig. 5). Generally, splinting is
hand function, prevent further wrist contracture
for ease of management, or address pain in the
wrist. As the fingers cannot be flexed tightly, there
is rarely a risk for breakdown of skin in the palm
of the hand.
Rigid splinting or casting are directed to
decreasing hypertonicity and increasing length in
wrist and finger flexors. These are best undertaken
for extended periods or over night. For patients in
whom the contracture of the wrist exceeds
a position of 45 of flexion, a series of two or
three casts over several weeks (Fig. 3) is recom-
mended. With this severe deformity, casting,
maintaining the joint for maximum time at end
range, has advantages over rigid splints that may
be uncomfortable.
A dorsal volar splint (Fig. 4) is used when there
is less contracture of the wrist and shortening of
FDS and FDP. This design is superior to other
splint designs because it uses an effective lever
system to apply an extension force to the wrist
and fingers [68]. Dorsal forearm and volar hand
components are remolded as lengthening occurs in
shortened tissues.
For people with this deformity, functional
goals often are determined by the competence of Fig. 3. Three plaster casts illustrate progressive increase
the other hand. For children with hemiplegia, the in range of passive wrist and finger extension with
goal may be to achieve an efficient gross grasp to successive applications.
580 J. Wilton / Hand Clin 19 (2003) 573–584

Pattern 4: wrist extension with intrinsic


hypertonicity
Spasticity in this pattern is located primarily in
the wrist extensor muscles, extensor carpi radialis
longus (ECRL) and extensor carpi radialis brevis
(ECRB), and hand intrinsic muscles, with adduc-
tion flexion at the thumb CMC and MCP, flexion
and adduction of finger MCP joints, and hyper-
extension deformities of the finger PIP joints.
With flexion of the MCP joints the intrinsic pull
on the lateral bands of the finger extensor
mechanism is facilitated across the dorsal aspect
Fig. 4. The dorsal volar splint has components for
dorsal hand and forearm, volar hand and fingers, and the
of PIP joint often restricting flexion motion at this
thumb. Each component is molded separately to position joint. The thumb metacarpal is held in an ad-
joints to address hypertonicity in wrist and finger ducted position by contraction in the AP and first
musculature. DI muscles, with spasticity in the flexor pollicis
brevis (FPB) contributing to MCP flexion and IP
extension.
Contracture can develop in the ECRL and
required to assist the wrist in achieving a more ECRB musculature, restricting wrist flexion. In-
neutral position to resolve the biomechanic trinsic muscle contracture is compounded by
disadvantage for FDS and FDP in grasp, while decreased extensibility of palmar fascia and skin,
not compromising EDC. Functional activities and by the fact EDC function is compromised in
requiring controlled finger opening and closing extremes of wrist extension (Fig. 6A). Failure to
complement the splinting program. manage contracture in the intrinsic muscles and
Functional splinting of the wrist may use rigid palmar tissues presents problems for skin care and
thermoplastic materials to immobilize the wrist in hygiene.
a position that does not compromise the ability to The major obstacle to functional use of the
extend the fingers during reach. Alternatively, hand is the inability to open the hand spontane-
materials such as Lycra or neoprene may be used ously. These patients need to disassociate elbow
to make circumferential soft splints, reinforced by extension from attempts to grasp, with opening of
splinting material or flexible boning that restricts the hand requiring a neutral wrist position and
wrist motion and opposes the wrist flexor spasticity. extension of finger MCP joints. In addition, the
Lycra or neoprene alone has insufficient strength thumb must be extended out of the palm of the
to oppose the strong flexion pattern and have no hand if any grasp is to be effective. In small
effect on extension deficits in the wrist or fingers. children with this pattern, the thumb MCP flexion
Splinting options for the wrist also should in- contracture often presents the most significant
corporate components to address the thumb de- problem, as the thumb is trapped constantly
formity as described in the previous classification. under the flexed fingers.

Fig. 5. (A) In the absence of active wrist extension in pattern 3 deformity, the wrist flexion deformity is reinforced by use
of the wheelchair control. (B) A rigid functional thermoplastic splint immobilizes the wrist to achieve a better
biomechanic position for finger and thumb musculature. A lining sock is worn under the splint.
J. Wilton / Hand Clin 19 (2003) 573–584 581

Fig. 6. (A) Pattern 4 deformity results in a posture of the hand with significant wrist extension and finger flexion, with
the thumb adducted and flexed across the palm. (B,C) A two-piece splint is required to control wrist and thumb positions
to allow active finger motion.

Therapeutic intervention 6B,C). The volar component is used to position the


Without intervention that has a long-term thumb in abduction and extension for opposition
perspective, this pattern of deformity can lead to to the fingers and to provide counterforce at the
significant contractures of the thumb and finger wrist. With appropriate splinting, finger extension
MCP joints. Treatment is directed toward pre- to activate switching or computer devices is
vention of intrinsic muscle contracture with main- possible, together with a simple gross grip.
tenance of tissue extensibility and restoration of Night splinting to address contracture in
the biomechanic balance to the wrist. shortened musculature and soft tissues requires
In young children who are highly motivated to the wrist to be positioned in some degrees of
use their hand, who can control their elbow flexion, the MCP joints in extension and abduc-
position, and who have no contractures, dynamic tion, the IP joints in some flexion, with main-
Lycra splints with design components to facilitate tenance of the thumb web space and MCP joint
wrist flexion and finger MCP extension can assist extension. Experience suggests that a reduction in
re-education of extrinsic–intrinsic muscle func- hypertonicity of the wrist and finger musculature
tion. Repetitive functional use of the hand is also improves elbow function.
essential for improved muscle strength. Gross
voluntary opening and closing of the fingers
around objects appropriate to the hand size and Pattern 5: fisted hand with wrist flexion or wrist
simple thumb–index finger pinch are appropriate extension
functional goals. Stretching protocols to prevent
This is the most severe deformity and reflects
contracture must address joint capsular structures,
the hypertonicity and secondary contracture of
particularly at the MCP joints and muscle tendon
muscles and associated connective tissues. The
unit length of the extrinsic finger musculature.
fingers and thumb are maintained in a fisted
In persons/people for whom tonal patterns are
position with minimal active motion evident. Skin
high and contracture is present, however, a greater
maceration and nail care are problems.
degree of wrist control is required, necessitating
more rigid functional splinting. Materials of choice
depend on whether the person can control wrist Therapeutic intervention
movement to neutral or slight flexion. Designs must The aim is to maintain sufficient motion so the
incorporate volar and dorsal components (Fig. hand can be opened to prevent maceration,
582 J. Wilton / Hand Clin 19 (2003) 573–584

infection, and breakdown of the skin of the palm neurodevelopment therapy plus casting and regular
and thumb. If opening of the fingers and occupational therapy for children with cerebral
positioning of the thumb are possible, casting is palsy. Dev Med Child Neurol 1991;33:379–87.
the preferred option. The gains are maintained by [5] Fetters L, Kluzik J. The effects of neurodevelop-
ment treatment versus practice on the reaching of
bivalving the final cast or applying a splint. If
children with spastic cerebral palsy. Phys Ther
casting is not an option, prefabricated palmar 1996;76:346–58.
protectors and custom made soft rolls can create [6] Kluzik J, Fetters L, Coryell J. Quantification of
an interface between tissue layers; however, they control: a preliminary study of effects of neuro-
do little to resolve contracture. This can be development treatment on the reaching of children
achieved by gradually adding firm components with spastic cerebral palsy. Phys Ther 1990;70:
made of splinting material to the soft rolls to hold 65–76.
joints more extended, with the circumference [7] Thibault A, Forget R, Lambert J. Evaluation of
determined by the degree of contracture in the cutaneous and proprioceptive sensation in children.
fingers and web space of the thumb. A reliability study. Dev Med Child Neurol 1994;
36:796–812.
[8] Yekutiel M, Jariwala M, Stretch P. Sensory deficit
Summary in the hands of children with cerebral palsy: a new
look at assessment and prevalence. Dev Med Child
The treatment of hand deformity and associ- Neurol 1994;36:619–24.
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Neurol 1999;41:586–91.
on many aspects of daily life. To assist therapists
[10] Krumlinde-Sundholm L, Eliasson A. Comparing
in analyzing patterns of movement of the wrist, tests of tactile sensibility: aspects relevant to testing
finger, and thumb musculature at rest and during children with spastic hemiplegia. Dev Med Child
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Rehabil 1993;74:602–9.
with CP and the highly individual functional [13] Dannenbaum RM, Lones LA. The assessment and
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is needed on the many possible interventions and ing cortical lesions. J Hand Ther 1993;6:130–8.
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[15] Katz RT, Rymer WZ. Spastic hypotonia: mecha-
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Hand Clin 19 (2003) 585–589

Pharmacologic management of the spastic and


dystonic upper limb in children with cerebral palsy
Stephen O’Flaherty, FRACP, FAFRM*, Mary-Clare Waugh,
FRACP, FAFRM
Department of Rehabilitation, Children’s Hospital at Westmead, Locked Bag 4001, Westmead,
Sydney NSW 2145, Australia

Cerebral palsy (CP) is a disorder of movement been shown to decrease consistently but tempo-
and posture that has its onset in the developing rarily spasticity in targeted muscles [5,6]. Whether
years. Spasticity is present in more than 80% of the child can use the relaxed state to improve their
individuals with CP. By definition, CP is a non- functional ability is still to be established. The
progressive, central nervous system deficit; how- authors’ experience with this product in the upper
ever, the musculoskeletal effects do change with limb has been positive and has reinforced the
time. importance of patient selection, the setting of goals
Spasticity or dystonia of the upper limb in that are relevant to the child, the careful verifica-
children with CP commonly is seen as part of tion of the needle location in the shoulder, arm,
a more involved clinical picture. Interventions for forearm, and hand muscles before injection, and
managing spasticity/dystonia need to be consid- the need for taking care of such details as pain-free
ered in this context and also in the context of each administration to ensure that the child’s cooper-
child’s goals, underlying motor function, degree ation with and confidence in the team is main-
of muscle contracture, ability to participate in tained [7]. The use of BTX-A in the upper limb is
therapy, and developmental level. The nonphar- described elsewhere in this edition. Alcohol and
macologic interventions used most frequently to phenol injections to manage spasticity in specific
assist in management include therapy (stretching, muscles are mentioned to highlight the number of
splinting, strengthening, positioning, and training practical problems and side effects that may occur
movement patterns [1]) and selective surgery, when used in children [8,9]. The focus of this article
including tendon/muscle releases and transfers is on the use of systemic medication to assist in
and dorsal rhizotomy. Thalamotomy, pallidot- managing the upper limb of children with severe
omy, and deep brain stimulation [2,3] may be spasticity or dystonia.
considered in patients with severe, disabling The three major subgroups of CP are diplegia,
dystonia whose symptoms of pain and discomfort hemiplegia, and quadriplegia. Depending on the
are not improved despite a full exposure to presence of spasticity or dystonia, affected chil-
pharmacologic (and other) interventions. dren are classified as spastic (dystonic) di-, hemi-,
The recent addition of botulinum toxin A and quadriplegic CP [10–12]. Most of the children
(BTX-A) to the pharmacologic options in the with severe generalized problems from spasticity
management of spasticity and dystonia makes it and dystonia fall within the quadriplegic classifi-
possible to target specific muscles in the upper limb cation group. This group is the most likely to
[4]. BTX-A has a rapid onset of action and has require systemic medication to help manage their
spasticity and dystonia. Children with spastic
* Corresponding author. diplegia now make up most of those newly
E-mail address: stepheno@chw.edu.au diagnosed with CP and, despite the implication
(S. O’Flaherty). in the name, diplegic children always have some
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00061-1
586 S. O’Flaherty, M-C. Waugh / Hand Clin 19 (2003) 585–589

involvement of their upper limbs that can be also are used to manage dystonia [18,22,23]. Mech-
relatively mild. The presence of a pseudobulbar anisms of action vary, but the result is either
palsy manifesting as dysphagia, drooling, and a suppression of muscle excitation or an enhance-
speech impairment would place children in the ment of neural inhibition. Response to medications
more severely affected (quadriplegic) group. is variable and not predictable before embarking
upon a clinical trial of the drug. As a result,
Spasticity treatment recommendations are not standardized
in the order that medication should be used and
Spasticity is a velocity-dependent increase in how medication combinations are prescribed.
muscle resistance to stretch that contributes to The principles of successful systemic pharma-
the impairment of function and reduced muscle cologic management are to determine [24]:
growth [13]. It is a major cause of disability in
the juvenile CP population.  whether a decrease in muscle hyperactivity
will cause any problems with function (eg,
Dystonia standing, reaching).
 whether it is the spasticity +/ÿ dystonia that
Children with dyskinetic CP may have this requires treatment.
in isolation or in combination with spasticity.  whether the goal is to improve function (eg,
Dyskinetic CP often manifests as athetosis or reach to a cup, use of a switch) or to assist
uncoordinated writhing movements in the young with care (eg, pain relief, hand hygiene, ability
child and then may evolve into a dystonic picture. to get the hyperflexed spastic elbow and wrist
Dystonia associated with cerebral palsy is through a sleeve). Use of medications such as
classified as secondary or symptomatic dystonia BTX-A for management of the elbow-flexed
and often is associated with abnormalities of the posture during effort are considered a func-
basal ganglia [14]. Dystonia is a movement dis- tional (as distinct from a cosmetic) goal.
order in which involuntary sustained or inter-  the goal the family should use to indicate
mittent muscle contractions result in twisting and a successful intervention such that appropri-
repetitive movements, abnormal postures, or both. ate dose rates can be ascertained (eg, im-
Despite its name children with dystonia may not provement in transferring, improvement in
have abnormal muscle tone at rest [15]. chair posture, reduction in spasm, sleeping
Dystonia has been described as the production longer).
of one pattern of muscle activity when a different  the results of side effects such as sedation and
pattern was intended [13]. Dystonia can be difficult seizure exacerbation in the child.
to recognize. The high variability of the speed of
contractions and co-contractions result in a con- No one medication has been found to meet all
tinual movement of affected muscle groups. The the above principles. Clinically we use medica-
dystonia disappears in certain positions (null tions on an open trial basis. The medications are
position) and in sleep, which distinguishes it from introduced slowly with printed guidelines to aid
tremor, myoclonus, and chorea [15]. the family. At times there can be dramatic positive
and negative effects. The listed medications (see
Tables 1 and 2) can have significant side effects
Pharmacologic management of spasticity
and this is often the limiting factor in their use.
and dystonia
When embarking on a trial of medication,
The aim of systemic pharmacologic manage- careful documentation of efficacy and tolerance of
ment is to reduce the muscle overactivity seen with the medication is necessary. Side effects need to be
spasticity and dystonia. Medication may be de- balanced against what the family and child see as
livered orally or through a feeding tube (enterally) positive responses.
or directly to the cerebrospinal fluid (intrathecally). When children have a mixed pattern of move-
There are four mainstream antispasticity enteral ment, treatment choices depend on the dominant
medications: baclofen, diazepam, dantrolene so- pattern of movement problem as a guide to which
dium, and tizanidine [16–21]. There are many other medication to trial first. Often multiple medica-
medications such as clonazepam and gabapentin tions are trialed serially or in combination, partic-
[17,18] that may have a role. The major antidysto- ularly for dystonias [25].
nia medications are L-Dopa and benzhexol (also Listed in Table 1 are the medications used in the
known as trihexyphenidyl). Baclofen and diazepam authors’ unit for significant generalized spasticity
S. O’Flaherty, M-C. Waugh / Hand Clin 19 (2003) 585–589 587

Table 1
Antispasticity and antidystonia medications and numeric ranking order of use
Antispasticity Order of use Antidystonia Order of use
Medication benefit for spasticity benefit for dystonia
Levodopa–Carbidopa — — 3 1
Benzhexol (trihexyphenidyl) — — 3 2
Baclofen 3 1 3 3
Diazepam 3 2 3 4
Tizanidine 3 3 — —
Dantrolene 3 4 — —
Intrathecal baclofen 3 After failure of 3 —
other measures
Carbemazepine — — 3 —
Clonazepam 3 — 3 —
Clonidine 3 — — —
Cyproheptadine 3 — — —
Gabapentin 3 — — —
Haloperidol — — 3 —
Tetrabenazine — — 3 —
3 Indicates that the medication has been used for this motor disorder.

and dystonia and a ranking of how these are of drugs have been used. The most common
introduced. The questions posed in the principles medications are levodopa-carbidopa, anticholi-
of management need to be considered at the outset nergics (benzhexol [trihexyphenidyl], benztro-
and when making changes to the child’s regimen. pine), baclofen, benzodiazepines (clonazepam,
Dosage and a summary of mechanism of action diazepam), dopamine depletors (tetrabenazine),
and major side effects are listed in Table 2. dopamine antagonists (haloperidol), and mexile-
Increased sedation, deterioration in seizure con- tine [15,22,31–35].
trol, and liver toxicity are the most significant side Response rates to medications vary widely.
effects that need to be considered. A full description Some children respond favorably to low-dose
of each of the medications is beyond the scope of benzhexol (4–6 mg/day) and others require high
this article and is covered extensively in Gracies doses (80 mg/day) before benefit is seen. In
et al 1997 [18] and Pranzatelli 1996 [26]. general children seem to tolerate higher doses of
Continuous infusion of baclofen by way of medications in comparison with adults who report
a catheter placed intrathecally and driven by an more intolerable side effects [36–39].
implanted programmable pump has been shown Dopa-responsive dystonia (DRD) may cause
to be effective in managing severe spasticity and a variety of motor symptoms that mimic other
dystonia [27,28]. It also has several potential disorders. It is recommended now that any child
complications [29]. A test dose is given as a bolus with unexplained dystonia should receive a trial of
or infusion to see if there is a significant, im- levodopa therapy [40,41]. If the child does have
mediate improvement in spasticity or dystonia. DRD, the response is often dramatic and further
This approach is more likely to allow assessment testing should be arranged. Most children with CP
of effect in children with severe dystonia and and dystonia, however, are not dopa-responsive
should be considered if other systemic agents are and this group is difficult to treat. Multiple medi-
ineffective (Table 2). cation trials including combinations of medica-
Treatment of dystonia is mostly on a symp- tions are often required [25,32].
tomatic basis. Focal dystonia in adults responds
best to BTX-A injections [30]. In children with CP
Summary
there are no reports of BTX-A treatment in
dystonia. The authors’ experience is that BTX-A Spasticity or dystonia of the upper limb in
can be beneficial particularly for painful muscle children with CP commonly is seen as part of
spasm from dystonia. a more involved clinical picture. Each can cause
Generalized dystonia can be managed with functional problems in and can interfere with the
enteral or intrathecal medications. A wide variety quality of life of children with CP. Pharmacologic
588 S. O’Flaherty, M-C. Waugh / Hand Clin 19 (2003) 585–589

Table 2
Medication, mechanism of action, initial and maximum dosages, and side effects
Drug Action Dosage: initial Dosage: maximum Side effects
Baclofen Stimulates GABA-b 5 mg/day for 1 80 mg/day total Drowsiness
receptor and suppresses week then Reduced seizure
excitatory neuro- increase by 5 mg threshold
transmitter release each week Gastro intestinal
TDS or QID track upset
Intrathecal As above 25 to 50 mcgm/day 1,000 mcg/day Pump and catheter
baclofen (constant infusion) problems
Diazepam Binds to GABA-a 2 mg/day 0.3–1 mg/kg/day Drowsiness Tolerance
receptors and increases Nocte BD or TDS
presynaptic inhibition
Dantrolene Inhibits calcium release 2 mg/kg/day 12 mg/kg/day Weakness Gastro
sodium from sarcoplasmic TDS or QID 100 mg QID intestinal tract upset
reticulum Sedation
Hepatotoxicity
Tizanidine Alpha 2 agonist decreases Adult dose 2–4 36 mg/day total Sedation
presynaptic activity in mg/day TDS Dizziness
excitatory neurons Hypotension
Dry mouth
Liver dysfunction
BTX-A Blocks release of — Varies according Weakness
acetylcholine at to preparation
neuromuscular junction
Benzhexol Anticholinergic 2 mg/day 80 mg/day total Dry mouth
(trihexyphenidyl) Nocte TDS or QID Blurred vision
Urinary retention
Levodopa- Dopaminergic 1 mg/kg/day 10 mg/kg/day Gastro intestinal
Carbidopa TDS or QID track upset
Insomnia
Clonazepam Enhances polysynaptic 0.5 mg 3 mg BD Sedation
inhibitory processes Nocte Drooling
Gabapentin GABA-ergic Anticonvulsant — Depression
dosages Sedation
Fatigue
Ataxia

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Dantrolene sodium: a review of its pharmacological hexyphenidyl in dystonia. Adv Neurol 1983;37:189–92.
properties and therapeutic efficacy in spasticity. [39] Hoon AH Jr, Freese PO, Reinhardt EM, Wilson
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[21] Meythaler JM, Guin-Renfroe S, Johnson A, Brun- dependent effects of trihexyphenidyl in extrapyrami-
ner RM. Prospective assessment of tizanidine for dal cerebral palsy. Pediatr Neurol 2001;25(1):55–8.
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Med Rehab 2001;82(9):1155–63. den CD. Successful treatment of childhood onset
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Hand Clin 19 (2003) 591–600

Botulinum Toxin A in the management of upper


limb spasticity in cerebral palsy
Terence Y.P. Chin, MBBSa,
H. Kerr Graham, MD, FRCS (Ed), FRACSa,b,*
a
Department of Orthopaedic Surgery, Royal Children’s Hospital, Melbourne, Flemington Road,
Parkville 3052, Victoria, Australia
b
University of Melbourne, Hugh Williamson Gait Analysis Laboratory, Royal Children’s Hospital, Melbourne,
Flemington Road, Parkville 3052, Victoria, Australia

Juvenile cerebral palsy (JCP) is the most skeletal muscle. An apt orthopaedic synonym for
common cause of the upper motor neurone cerebral palsy is short muscle disease [6].
syndrome (UMNS) in children and is associated In spastic hemiplegia, the upper limb deform-
with positive and negative features [1–3]. The ities typically include internal rotation and adduc-
positive features are movement disorders, such as tion at the shoulder, and this may progress in
spasticity, dystonia, athetosis, and co-contraction. a small minority of patients to anterior migration
The negative features are weakness, dyspraxia, of the humeral head. The pectoralis major and the
impaired selective motor control, and sensory subscapularis are usually the main deforming
impairment. Physicians and therapists tend to forces. At the elbow, flexion deformity is common,
focus on the positive features of UMNS (spastic- initially dynamic, progressing to a fixed deformity
ity and contractures) because these are influenced with variable degrees of joint contracture. In the
more easily by intervention than are the negative forearm, pronation deformities are ubiquitous and
features. The negative features, however, largely are the result of muscle imbalance between spastic
determine the long-term functional prognosis. pronators (pronator teres and pronator quadra-
There is no satisfactory solution to weakness tus) and weak supinators. Loss of active supina-
and lack of sensibility. tion results in a contracture in the interosseous
Associated comorbidities include cognitive membrane and may predispose to subluxation or
impairment, learning difficulties, epilepsy, respi- dislocation of the radial head. Flexion deformities
ratory disease, and gastrointestinal problems. of the wrist and fingers are the most common
These problems are more common in children deformities in the upper limb, because the spastic
with severe cerebral palsy, especially children with flexors overpower the weaker extensors. Wrist
spastic quadriplegia. flexion deformity usually is accompanied by ulnar
deviation, because the ulnar deviators are more
Neuromusculoskeletal pathology powerful than the radial deviators. The ‘‘thumb-
in-palm’’ deformity is common and is associated
At birth the child with JCP does not have fixed with significant functional impairment [5,6].
deformities. These are acquired during growth As with all deformities, there is a postural-
[4,5]. The key feature of the musculoskeletal dynamic phase with gradual development of fixed
pathology is a failure of longitudinal growth of contracture of the muscle–tendon units caused
by muscle imbalance. Eventually this leads to the
* Corresponding author. phase of decompensation, which may include fixed
E-mail address: kerr.graham@rch.org.au joint contractures and joint subluxation. Different
(H.K. Graham). muscles develop fixed contractures at different
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00060-X
592 T.Y.P. Chin, H.K. Graham / Hand Clin 19 (2003) 591–600

speeds. The pronator teres is invariably the first obic conditions. It binds to cholinergic nerve
muscle in the hemiplegic upper limb to develop endings and inhibits release of the neurotransmit-
a contracture. This is not because it is more spastic ter acetylcholine by blocking the binding of
than other muscles but because it is never stretched acetylcholine vesicles to the interior of the motor
by antagonist action [5,7]. Loss of active supina- end-plate plasma membrane. The sprouting of
tion is present to some degree in all children with new nerve endings restores neurotransmission. It
hemiplegia. Prevention of pronation contracture is takes approximately 3 months for the original
simple in principle but difficult in practice. The nerve endings to be restored while the ‘‘new’’
pronator teres must be stretched out fully to its nerve endings are being eliminated concurrently
maximum length by parent or therapist, because [11]. The effect of BoNT-A thus is pharmacolog-
the child is unable to do this for himself. ically completely reversible.
Of the six serotypes, only types A and B have
become commercially available. Type B has
Spasticity management in juvenile cerebral palsy
a shorter duration of action than type A and offers
Spasticity management has become increas- no advantages in the management of chronic
ingly sophisticated and effective in recent years and spasticity associated with JCP. There are two
is best managed by a multidisciplinary team. The commercially available type A preparations, Dys-
spasticity team ideally should have representation portÒ (Ipsen; UK) and BotoxÒ (Allergan; USA).
from developmental pediatrics, neurology, phys- Although they are both type A neurotoxins, the
iotherapy, occupational therapy, neurosurgery, preparations differ significantly in preparation,
plastic surgery, and orthopaedic surgery. potency, and antigenicity [12]. The units are not
Spasticity interventions can be classified as interchangeable and clinicians must be vigilant in
temporary or permanent and as focal or general- calculating the appropriate dosage [8]. The ap-
ized [7,8]. The choice of appropriate spasticity proval and licensing of the commercially available
management often is dictated by the topographic neurotoxins vary from country to country. BotoxÒ
distribution of cerebral palsy and associated and DysportÒ are approved in Australia and most
comorbidities. In spastic monoplegia and hemi- European countries for the management of spastic
plegia, focal or regional spasticity management equinus in children with cerebral palsy, but the
with injections of Botulinum Toxin A (BoNT-A) approval does not extend to the injection of spastic
to the appropriate target muscles is a widely used upper limb muscles. In the United States, the
intervention. In spastic diplegia, selective dorsal Federal Drug Administration (FDA) has ap-
rhizotomy may be indicated for lower limb proved neither preparation for use in children
spasticity. There may be incidental improvements with cerebral palsy. Upper limb injections in
in upper limb function after rhizotomy in the spastic cerebral palsy are therefore an ‘‘off label’’
lumbosacral area [9]. In spastic quadriplegia, indication. The authors believe that all ‘‘off label’’
continuous intrathecal administration of baclofen use of BoNT-A should be in the context of ethically
by implantable pump may be appropriate. Signif- approved and properly regulated clinical trials.
icant reduction in upper limb spasticity and When injected into skeletal muscle, BoNT-A
improvements in function can be achieved in some causes a dose-dependent, reversible chemodener-
children by placing the tip of the intrathecal vation of muscle [13]. The treatment converts
catheter high enough to achieve a significant paresis with muscular hyperactivity to paresis with
concentration of baclofen in the cerebrospinal fluid muscular hypoactivity [14]. In general BoNT-A
(CSF) at the level of the cervical roots [10]. should be considered a focal/regional intervention
Interventions for the management of spasticity [8,10].
can be combined when appropriate. In teenagers Koman et al in the United States, Wall et al in
with spastic quadriplegia, global spasticity man- South Africa, and Graham et al in the United
agement may require intrathecal baclofen. Re- Kingdom reported the first clinical trials of in-
sidual elbow flexor spasticity can be managed by tramuscular BoNT-A in cerebral palsy in 1993 and
focal injection of BoNT-A. 1994 [13,15,16]. The authors first investigated the
effects of BoNT-A injections in an animal model of
JCP, the hereditary spastic mouse. The authors
Intramuscular botulinum toxin A
found that injections of BoNT-A to the gastrocso-
BoNT-A is a potent neurotoxin produced by leus muscle in juvenile mice improved growth of
the bacterium Clostridium botulinum under anaer- the muscle–tendon unit and reduced contracture
T.Y.P. Chin, H.K. Graham / Hand Clin 19 (2003) 591–600 593

[4]. In an open label clinical study, the authors and some improvements in function followed
found that injections of spastic lower limb muscles reduced muscle stiffness. The strengths of the
in JCP resulted in reduced spasticity, improved authors’ study were that it was double blind and
joint range of motion, and improved gait and placebo controlled and that an objective measure
function [16]. Improvements in equinus gait were of muscle stiffness was included. The weaknesses
confirmed in a subsequent randomized clinical were that the study cohort was small and hetero-
trial by the authors’ group and replicated in other geneous, the amount of occupational therapy was
centers [17,18]. The authors then designed a ran- not controlled or recorded, and the study lacked
domized, placebo-controlled clinical trial to in- a good functional outcome measure. Most of these
vestigate the effects of intramuscular BoNT-A in shortcomings have been addressed in a more recent
the upper limb of children with cerebral palsy [19]. study by Fehlings et al [21]. They randomized 30
The authors’ study was placebo controlled and children with hemiplegic JCP to receive occupa-
double blind. Children were assigned randomly to tional therapy (control group) or occupational
receive multiple injections of BoNT-A or normal therapy combined with intramuscular injections of
saline to selected muscle groups in the upper limb. BoNT-A. In this larger randomized clinical trial
The child, parents, therapists, and investigators they demonstrated significant functional gains
were blind to the treatment group until the code using the Quality of Upper Extremity Skills Test
was broken 12 weeks later. Extensive testing of (QUEST), a validated tool measuring upper limb
muscle stiffness, joint range of motion, and function [22]. This study has been critical to the
function was performed at baseline and at 2 and development of good study design for upper limb
12 weeks after injection. The main results were that trials in JCP. The need for a control group is made
intramuscular injection of BoNT-A resulted in clear by the demonstration of progressive improve-
consistent reduction in spasticity and muscle ment in function in the control group, probably
stiffness as measured by a torque motor (Fig. 1). because of natural improvement with time and the
The torque motor was used to measure the acquisition of functional skills. Validated measures
resonant frequency of the wrist as an objective such as QUEST and the Melbourne upper limb
and quantifiable measure of forearm muscle assessment scale should be used to quantify
stiffness [6,20]. A reduction in spastic posturing, functional outcomes of spasticity management
improved active and passive joint range of motion, objectively [22,23].

Fig. 1. Change in forearm muscle stiffness as measured by wrist resonant frequency (Hz2) following injection of BoNT-A
into forearm flexors in a child with spastic hemiplegia. A dramatic decline in forearm muscle stiffness from 20.7 Hz2 to an
almost normal value of 5.6 Hz2 is seen at 1 week after BoNT-A injection into wrist flexors. Subsequent measurements
show a gradual increase in muscle stiffness approaching (but still lower than) baseline levels at 1 year. Repeat BoNT-A
injection produces a second fall in muscle stiffness, albeit to a lesser degree compared with the first injection. Note that
although the pharmacologic effect of BoNT-A lasts for 3–6 months, the biomechanical benefits may persist up to 1 year.
(From Corry IS, Cosgrove AP, Walsh EG, McClean D, Graham HK. Botulinum toxin A in the hemiplegic upper limb:
a double blind trial. Dev Med Child Neurol 1997;39:185–93; with permission.)
594 T.Y.P. Chin, H.K. Graham / Hand Clin 19 (2003) 591–600

Treatment goals sponse to weakening of the elbow flexors, wrist


flexors, and finger flexors, particularly in the
Intramuscular injections of BoNT-A can be
presence of dynamic contractures alone [24]. The
used for a variety of indications in the upper limbs
presence and efficacy of extensor and supinator
of children with cerebral palsy. The most common
function, previously masked, can be assessed.
indication is for the correction of spastic posturing
Severe postoperative pain and spasm may
that interferes with function in the absence of fixed
complicate muscle–tendon surgery in many chil-
deformity [16,19]. Dynamic deformities are caused
dren with spastic cerebral palsy. BoNT-A has been
by dystonia or spasticity and are absent under
found to provide effective pain relief in a double
anesthesia. Fixed deformities are found in the
blind, placebo-controlled trial [26]. Reduction in
older child and require muscle–tendon surgery for
spasm also protects lengthened tendons and
correction. Determining the effect of dynamic
tendon transfers from postoperative disruptions.
deformities on function can be difficult, but it
is a vital step in predicting whether functional
benefits can be obtained. The transition from Selection of target muscles for BoNT-A injection
dynamic to fixed deformity in many children is
The selection of target muscles for BoNT-A
gradual. Dynamic and fixed deformities coexist in
injection is individualized after a careful analysis of
many children. It requires considerable judgment
the functional deficits, the postural deformities, the
to decide when to use BoNT-A alone and when to
treatment goals of the child and family, and the
use combinations of BoNT-A followed by serial
clinician’s analysis of the muscular hypertonia and
casting for residual contracture. In general, BoNT-
how it relates to the individual’s abilities and
A is more appropriate in the younger child with
disabilities [19,21]. This is a complex process re-
dynamic deformities and muscle–tendon surgery
quiring experience and time. A systematic ap-
is reserved for the older child with fixed deform-
proach is important, starting with patient/family
ities. Those children with mild spasticity and the
identified limitations, problems, and goals. This is
inability to initiate voluntary finger movements are
followed by objective assessment of the movement
also less likely to benefit. Patients need to be able
disorder and characteristic posturing at rest and
to activate and strengthen antagonist muscles to
during functional activities. Selective motor con-
take advantage of BoNT-A weakened agonists.
trol and active and passive joint range of motion
Those with a good baseline grip strength are more
should be assessed by standardized examination
likely to benefit, as muscle strength and spasticity
techniques and should be recorded. Functional
are reduced after injection [24]. Other favorable
testing using assessments such as the Melbourne
factors are: young age, good capacity for motor
Upper Limb Assessment is strongly recommended,
learning, and high levels of motivation [25].
together with video recording of specific postural
The correction of spastic posturing has a sig-
and functional tasks. Upper limb kinematics and
nificant impact on deformity and self esteem.
dynamic electromyography require specialized
When the authors investigated the functional
equipment, expertise, and time. They are valuable
outcomes of BoNT-A injections in the hemiplegic
in a research setting but are not yet widely available.
upper limb, the outcome that was rated most
Spastic postures should be identified and the
highly was reduction in elbow flexor posturing
degree of spastic and fixed shortening of all the
when running, a cosmetic rather than a functional
major muscle tendon units should be documented.
outcome [19]. In severely affected patients for
The retained function must be identified and
whom functional improvements are not achiev-
documented, that is, the voluntary control of
able, BoNT-A still may be used to improve spastic
individual muscle–tendon units and muscle
posturing, enabling easier management of hy-
strength. In the authors’ practice the most common
giene, dressing, and other aspects of general care.
target muscles, in descending order of frequency,
Additional indications for BoNT-A therapy
have been:
include preoperative planning and perioperative
pain and spasm control. Preoperative BoNT-A  Pronator teres (93%) and pronator quadratus
chemodenervation can be used to simulate sur- (2.9%)
gery. In the presence of fixed contracture, a  Wrist flexors: flexor carpi ulnaris (86%) and
complete surgical simulation is not possible. It flexor carpi radialis (82%)
can be useful, however, for the child, the family,  Elbow flexors: biceps (78%), brachialis
and the therapists to determine the child’s re- (72%), and brachioradialis (6%)
T.Y.P. Chin, H.K. Graham / Hand Clin 19 (2003) 591–600 595

 Adductor pollicis and flexor pollicis brevis quired for this (Fig. 3). Other frequently used
(76%) methods to locate muscles accurately include
 Long finger flexors (31%) and flexor pollicis electromyography (EMG) and muscle stimulation.
longus (43%) EMG works well in cooperative adult patients, but
 Pectoralis major (16%) and subscapularis direct stimulation is the authors’ preferred tech-
(12%) nique for children [27]. Stimulation is achieved by
using an insulated needle for stimulation of the
When the aim is to decrease typical spastic
muscle and injection of the toxin into the target
posturing and to improve function, the usual
muscle belly (Figs. 4–6) [27]. For most children this
combination is biceps and brachialis, pronator
requires mask anesthesia [19].
teres, flexor carpi ulnaris, flexor carpi radialis, and
In children, BoNT-A doses are usually on a unit/
adductor pollicis. The long finger flexors should be
muscle/Kg of body weight basis. The two com-
avoided carefully to prevent weakening grip
mercially available preparations of type A toxin,
strength. When the aim is improved palmar
BotoxÒ and DysportÒ, have different potencies
hygiene, injection of the finger flexors (flexor
and the units are not interchangeable. The type B
digitorum superficialis and profundus) is always
toxin has a shorter duration of action, greater
required.
systemic spread, and more side effects than the
type A toxins [12]. The only logical indication for
Location of the target muscles and dose levels type B toxin is when antibody-mediated resistance
to type A toxin develops following repeated
Target muscles are identified by simple means BoNT-A administration.
such as palpation and the use of anatomic land- Current dose guidelines have been established
marks (Fig. 2). This may be adequate for large empirically by consensus of experienced injectors
subcutaneous muscles such as the biceps but is and have been described fully elsewhere (Table 1)
not reliable for smaller, deeper muscles. BoNT-A [25,28,29]. The dose required is based on the
diffuses readily across fascial barriers and the number of selected target muscles, the degree of
effects of injection of neurotoxin into one muscle hypertonia, and the desired effect.
may be complicated by diffusion into another
muscle or even into another compartment. It is not
known how the location and number of injection
Injection aftercare
sites and the dose of toxin and its dilution influence
the diffusion characteristics. Intuitively it makes The first episode of upper limb injections is
sense, however, to locate target muscles as a clinical experiment and close follow-up is
accurately as possible. A good understanding of required to describe the response. The first post-
longitudinal and cross-sectional anatomy is re- injection assessment should be at 2–3 weeks after

Fig. 2. Typical pattern of spastic flexion deformity of the upper limb in a patient with CP. Sites for injection of BoNT-A
are illustrated: biceps brachii for elbow flexion, flexor carpi radialis and flexor carpi ulnaris for wrist flexion, pronator
teres for forearm pronation, and adductor pollicis for thumb-in-palm posturing.
596 T.Y.P. Chin, H.K. Graham / Hand Clin 19 (2003) 591–600

Fig. 3. Longitudinal and cross-sectional anatomy of the upper limb with common target muscles shaded in cross-
section. A good knowledge of this anatomy is essential for accurate needle placement for BoNT-A injections.

injection, which is the time of peak pharmaco- and occupational therapy should be instituted
logic effect. The active and passive joint range of when appropriate to improve range of motion,
motion benefits, unintended effects, and adverse strengthen antagonist muscles, and improve pat-
events are documented. Need for splintage and terns of functional movements (eg, grasp and
casting can be determined at this stage. Physical release, pincer grip). Functional gains may take

Fig. 4. Equipment for BoNT-A injection. Shown are a vial of BotoxÒ (Allergan, USA), normal saline for reconstitution,
insulated injection needle, and a portable electrical stimulator.
T.Y.P. Chin, H.K. Graham / Hand Clin 19 (2003) 591–600 597

Fig. 5. BTX-A injection into pronator teres. The patient’s forearm is placed initially in full supination. Following needle
placement, electrical stimulation produces pronation of the forearm, confirming needle placement into the muscle belly
of pronator teres. Injection sites for biceps brachii, FCR, and FCU also are marked.

longer to become evident and a repeat assessment dystonic/spastic cerebral palsy. Most children had
of function should be deferred until approxi- spastic hemiplegia, but there were 12 children with
mately 3 months after injection. spastic quadriplegia who had bilateral upper limb
involvement and bilateral injections of BoNT-A.
In more than one third of children with hemiplegia,
Results and complications
upper limb injections were combined with lower
Between 1994 and 2002, the authors injected the limb injections for spastic equinus deformity. The
upper limbs in 103 children with spastic or mixed most common indication for BoNT-A in the upper

Fig. 6. BoNT-A injection into adductor pollicis. An assistant holds the first web space fully abducted as the needle is
placed into the muscle belly of adductor pollicis. Needle placement is confirmed with electrical stimulation.
598 T.Y.P. Chin, H.K. Graham / Hand Clin 19 (2003) 591–600

Table 1
Botulinum toxin A: upper limb sites and dose guidelines (Botox only) for children with spastic hemiplegia
Number of
Clinical pattern Muscles involved Botox dose U/Kg injection sites
Pronated forearm Pronator teres 1 1
Pronator quadratus 1 1
Flexed elbow Biceps 2–3 4
Brachialis 2 2
Brachioradialis 1 1
Flexed wrist Flexor carpi ulnaris 1–2 1
Flexor carpi radialis 1–2 1
Thumb-in-palm Adductor pollicis/flexor 5–10 Ua 1
pollicis brevis
Flexor pollicis longus 10–20 Ua 1
Clenched fist Flexor digitorum profundus 1–2 4
Flexor digitorum superficialis 1–2 4
Adducted/internally Pectoralis major 2 2–3
rotated shoulder
Subscapularis 1 1–2
a
Total dose per muscle.
References: [25,28,29].

limb was improvement in function (Table 2). The is evidence of a systemic spread of toxin and we
most common adverse event was weakness of have reported previously three cases of inconti-
grasp, which was noted in 12 children (11.7%) nence after lower limb injections [30].
(Table 3). This resolved fully in all children by The outcome of BoNT-A injections to the
3-month follow-up. Bruising and pain at the upper limb in children with cerebral palsy can be
injection site was found in 14 children (13.6%). classified according to the scheme the authors
Three children experienced a ‘‘dropped index devised for lower limb injections [30]. In approx-
finger.’’ This lasted 2–6 weeks before resolution imately 3%–5% of children a dramatic improve-
occurred. One child had urinary and fecal in- ment in posture and function is evident and the
continence after combined upper and lower limb results are long-lasting, in some cases permanent.
injections at a total dose of 12 U/Kg BotoxÒ. The authors have referred to this group as ‘‘golden
Incontinence developed within 2 days of injection responders.’’ The authors are unsure as to the
and resolved after 3 weeks. The authors believe this mechanism of the response. Peripheral biome-
chanic factors and central mechanisms may be
responsible. The authors currently are investigat-
Table 2
ing central and peripheral effects after injections
Indications for upper limb BoNT-A and percent re-
sponse rate in 103 children with cerebral palsy
of BoNT-A in a randomized clinical trial. Most
children (approximately 70%) demonstrate a clin-
Number and percent ical or symptomatic response that is unfortunately
Total of golden or
number clinical respondersa
Dynamic upper 62 41 (66%) Table 3
limb function Adverse events in 103 children who received botox
Presurgical trial 14 12 (86%) injections to the upper limb for the management of
Combined with upper 12 — spasticity
limb surgery
Number Percent
Hygiene/splinting 12 11 (92%)
Painful arm spasms 3 3 (100%) Weakness of grasp 12 11.7
Total 103 67 of 91b (74%) Bruising 10 9.7
a Pain 4 3.9
See text for classification of responders after BTX-
Dropped index finger 3 2.9
A injection.
b Incontinence 1 1.0
Total of 91 patients after exclusion of 12 who had
Total 30 29
combined BoNT-A and upper limb surgery.
T.Y.P. Chin, H.K. Graham / Hand Clin 19 (2003) 591–600 599

relatively short lived (6–9 months) (Table 2). The photography and illustrations. We also thank
authors refer to this group as ‘‘clinical respond- Mary Sheedy for assistance with the preparation
ers.’’ A reduction in muscle tone is observed, of this manuscript.
accompanied by improved joint range of motion
and some small gains in function. The decision
whether to reinject and to continue with a combi- References
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and their parent terminals. Proc Natl Acad Sci USA
The authors wish to thank the staff of the 1999;96:3200–5.
Educational Resource Centre of the Royal Child- [12] Aoki KR, Guyer B. Botulinum toxin type A and
ren’s Hospital, Melbourne, for their help with other botulinum toxin serotypes: a comparative
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review of biomechanical and pharmacological [22] ICIDH-2. International Classification of Impair-


actions. Eur J Neurol 2001;8(Suppl 5):S21–9. ments, Disabilities and Handicaps—beta draft 2.
[13] Wall SA, Chait LA, Temlett JA, Perkins B, Hillen World Health Organization, Geneva, Switzerland.
G, Becker P. Botulinum A chemodenervation: a new Address at: http://www.whosis/icidh/index.html.
modality in cerebral palsied hands. Br J Plast Surg [23] Randall M, Johnson L, Reddihough D. The
1993;46:703–6. Melbourne assessment of unilateral upper limb
[14] Berweck S, Graham HK, Heinen F. Spasticity in function. Melbourne: Arena Printing, Royal Child-
children. In: Moore P, Naumann M, editors. ren’s Hospital; 1999. p. 80.
Handbook of botulinum toxin treatment. 2nd [24] Fehlings D, Rang M, Glazier J, Steele C. Botulinum
edition. Oxford: Blackwell Science Ltd; 2003. toxin type A injections in the spastic upper
p. 272–305. extremity of children with hemiplegia: child char-
[15] Koman LA, Mooney JF III, Smith BP, Goodman acteristics that predict a positive outcome. Eur J
A. Management of cerebral palsy with Botulinum Neurol 2001;8(Suppl 5):S145–9.
toxin A: report of a preliminary randomised, double [25] Autti-Rämö I, Larsen A, Taimo A, von Wendt L.
blind trial. J Pediatr Orthop 1994;14:229–303. Management of the upper limb with botulinum
[16] Cosgrove AP, Corry IS, Graham HK. Botulinum toxin type A in children with spastic type cerebral
toxin in the management of the lower limb in cerebral palsy and acquired brain injury: clinical implica-
palsy. Dev Med Child Neurol 1994;36:386–96. tions. Eur J Neurol 2001;8(Suppl 5):S136–66.
[17] Corry I, Cosgrove A, Duffy C, McNeil S, Taylor T, [26] Barwood S, Baillieu C, Boyd RN, Brereton K, Low
Graham H. Botulinum toxin A compared with J, Nattrass G, et al. Analgesic effects of botulinum
stretching casts in the treatment of spastic equinus: toxin A: a randomized placebo-controlled clinical
a randomised prospective trial. J Pediatr Orthop trial. Dev Med Child Neurol 2000;42:116–21.
1998;18:304–11. [27] O’Brien CF. Injection techniques for Botulinum
[18] Flett PJ, Stern LM, Waddy H, Connell TM, Seeger toxin using electromyography and electrical stimu-
JD, Gibson SK. Botulinum toxin A versus fixed cast lation. Muscle Nerve 1997;20(Suppl 6):S176–80.
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palsy. J Paediatr Child Health 1999;35:71–7. palsy: a rational approach to a treatment protocol,
[19] Corry IS, Cosgrove AP, Walsh EG, McClean D, and the role of botulinum toxin in treatment.
Graham HK. Botulinum toxin A in the hemiplegic Muscle Nerve 1997;20(Suppl 6):181–91.
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Neurol 1997;39:185–93. Delgado MR, Gaebler-Spira DJ, et al. Recommen-
[20] Walsh EG. Muscle masses and motion: the physi- dations for the use of botulinum toxin type A in the
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125). London: Mac Keith Press; 1992. p. 220. [30] Boyd RN, Graham JEA, Natrass GR, Graham
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improve upper extremity function in children with management of spasticity in children with cerebral
hemiplegic cerebral palsy. J Pediatr 2000;137:331–7. palsy. Eur J Neurol 1999;6(Suppl 4):37–45.
Hand Clin 19 (2003) 601–606

Electrical stimulation in the management


of spastic deformity
Luis R. Scheker, MDa,*, Kagan Ozer, MDb
a
Division of Plastic and Reconstructive Surgery, University of Louisville School of Medicine,
225 Abraham Flexner Way, Suite 800, Louisville, KY 40202, USA
b
C.M. Kleinert Institute for Hand and Microsurgery, 225 Abraham Flexner Way,
Suite 850, Louisville, KY 40202, USA

Cerebral palsy (CP) in children continues to be in the management of patients with CP. One
a severe problem, with an incidence of 2–5 per such treatment is functional electrical stimulation
1000 people in developed countries [1–4]. Half (FES).
have upper extremity involvement [5]. Traditional Levine in 1952 first introduced the concept of
treatment methods consist of surgery, physio- electrical stimulation in the management of CP
therapy, orthoses, and medication. Evidence for [19]. Treatment consisted of stimulation of the
the efficacy of these treatment modalities in the antagonist muscles to oppose a spastic muscle
literature is scarce and often confounded by the group, followed by vigorous ROM exercises,
problems of extensive variability in the clinical which led to a dramatic decrease in the muscle
presentation and types of management offered tone. Alfieri also demonstrated a long-term re-
and difficulty in objective assessment of outcomes. duction of muscle tone by administering multiple
Surgery is performed to rebalance the muscles in treatment sessions of FES to the antagonist of
the limb to improve function, to decrease hygiene spastic muscles [20]. Later the technique found
problems, and to improve cosmesis [6]. Some a more common application in the lower extrem-
patients with CP, however, have contraindications ities [21]. There have been few studies on its use on
for upper extremity surgery [4], and even when the upper limb affected by CP. In 1991, the first
appropriate, surgery may fail to improve function. report of FES being used for the upper limbs of
The amount of conclusive research supporting children with CP was published [22]. The study
the use of physical therapy to improve function involved two children who received FES to their
is limited [7]. The application of orthoses in wrist extensors and demonstrated the feasibility of
the upper extremity is common but results have application without a significant improvement in
seldom been reported [8–11]. Medications to function. Later, Carmick reported three patients
paralyze the spastic muscle group have major side with improved grasp and release activities and
effects of sedation and weakness [12–15]. Recent locomotor efficiency after applying FES to vari-
studies on Botulinum toxin A showed encourag- ous spastic and nonspastic muscle groups [23,24].
ing results in relieving spasticity and increasing The success of this treatment was attributed to
range of motion (ROM), with no improvement in strengthening of spastic and nonspastic muscle
fine motor skills and no carryover effect after groups, increased awareness, and spontaneous use
3 months [16–18]. Investigators therefore con- of the upper limb. Following these anecdotal
tinue to look for alternative treatment modalities reports, the authors published our experience with
neuromuscular electrical stimulation (NMES)
together with dynamic bracing in 1999 [25]. This
* Corresponding author. preliminary report included 19 patients, some with
E-mail address: lscheker@kkahand.com more than 5 years of follow-up, this being the
(L.R. Scheker). largest series with longest follow-up in the
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00037-4
602 L.R. Scheker, K. Ozer / Hand Clin 19 (2003) 601–606

literature. The authors’ preliminary results Patient selection criteria


showed remarkable improvement in hand func-
Between 1992 and 1997, children visiting The
tion, ROM, and cosmesis. Finally, Wright, in his
Kentucky Commission for Children with Special
report of eight cases, used FES and demonstrated
Health Care Needs with a diagnosis of spastic
significant improvements in hand function and
hemiplegic CP who met the following additional
wrist extension until the end of follow-up (6
criteria were considered eligible for the study:
weeks) [26].
(1) patients who were 3 years of age and older
Children with CP have motor, sensory, and
(3–21 years), (2) patients with mild to moderate
intellectual problems that should be evaluated
spasticity of the shoulder and elbow that allowed
together. In children with a spastic-type upper
the patient to place the hand in a desired position
extremity involvement, the elbow is usually in
in space, (3) patients with protective sensation, (4)
flexion and the forearm in pronation; the wrist is
patients with cognitive abilities that enabled them
in ulnar deviation and flexion, with variable
to follow doctors’ directions, and (5) patients with
extension ability; the thumb is adducted and
moderate to severe spasticity of the wrist and
flexed into the palm [4]. Excessive wrist and elbow
hand without any static contracture.
flexion and forearm pronation do not allow
The Zancolli classification was used to grade
manipulation of objects. Release is typically slow,
the patients before and after the application of the
with excessive wrist flexion instead of extension,
protocol (Table 1) [32]. Nineteen patients were
caused by the inability to extend the fingers and
evaluated before the treatment: 11 were classified
wrist at the same time. Wrist flexion mechanically
at type III, 7 as type IIb, 1 as type IIa. No one was
assists finger extension using the tenodesis effect
classified as type I.
of the extensor digitorum communis (EDC) muscle
as the wrist is flexed. Motion is limited because of
decreased strength of the extensors, contractures, Electrical stimulation
and the increased muscle tone of the flexors
[6,27,28]. The disease is associated with positive The electrical stimulation system is comprised
and negative features. Spasticity and hyperactive of three elements: the stimulator unit (EMS 400
reflexes are considered positive features. Muscle Skylark Device Co., Louisville, Kentucky), elec-
weakness and loss of motor control are considered trodes, and connecting wires (Medi-Stim Inc.,
negative features. In the past, spasticity was Delaware, Ohio). The reusable, self-adhering,
considered the most disabling factor. Currently, carbonized rubber electrodes are connected to
many researchers consider the negative symptoms the stimulator by leads that are snapped to the
(muscle weakness, loss of control) more disabling button of the electrode. The adhesive electrodes
[29,30]. Function seems to correlate with strength,
not with muscle tone [31].
Table 1
The authors’ approach in the management of
Zancolli classification of wrist and finger deformity
CP consists of the combined use of NMES of
nonspastic extensors of the wrist together with the Before the
use of dynamic (daytime) and static (nighttime) Zancolli classification protocol Follow-up
bracing. Using NMES, the authors stimulate the Type 1: Complete extension None 10
nonspastic extensor compartment muscles, help- of the fingers with wrist
ing them to regain their strength and possibly to in neutral position or with
stimulate a retrograde reflex arc inhibiting the less than 20 flexion
Type 2a: Active extension 1 9
spastic contracture of the flexors. The dynamic
of the fingers with
brace used in the authors’ protocol only allows
wrist flexion of greater
active metacarpophalangeal (MP) joint flexion than 20 , active wrist
and fixes the wrist joint in 10 dorsiflexion. This extension present
type of bracing helps the effective stretching of Type 2b: Active extension of 7 None
the spastic flexors without stimulating spastic the fingers with wrist flexion
contraction and assists in shortening of the of greater than 20 , active
extensor musculature while strengthening intrin- wrist extension absent
sic muscles. The following text includes a detailed Type 3: No active extension 11 None
description and the results of the authors’ new of the fingers even with
maximal wrist flexion
approach.
L.R. Scheker, K. Ozer / Hand Clin 19 (2003) 601–606 603

are placed on the dorsum of the forearm over the phalangeal (DIP) joints in the extended position,
bellies of the wrist and finger extensor muscles at allowing finger movement only at the MP joints.
distal and proximal positions (Fig. 1). To resist the spastic muscles but not facilitate
The battery operated electrical stimulator a spastic response (that is, a stretch reflex), the wrist
consists of a dual-channel with a current output unit is positioned 10 short of maximum extension
that can be set between 0 and 100 mA. The stretch with the finger platform immobilizing the
stimulus waveform consists of biphasic symmetric IP joints, allowing MPJ motion. The elbow unit
rectangular pulses with a 200-lsec duration. The has a dynamic dual hinge with adjustable tension
pulse rate ranges from 40–60 pulses per second to (0–7.2 kg) and an adjustable lock.
produce tetanic muscle contraction. The stimulus This stretching technique resists tight spastic
amplitude is adjusted from 30–40 mA to produce flexors while stretching intrinsic musculature and
tolerable muscle contractions. During the training gives the antagonist musculature the chance to
sessions with the parents, electrical stimulation is shorten. The custom-fitted orthosis (Ultraflex;
applied first on the clinician’s arm, then on the Malvern, Pennsylvania) is used to alter the elbow
arm of the parents, and finally on the patient’s flexion contracture and pronation deformity by
arm. A 2-second ON ramp, 2-second OFF ramp, placing the forearm in maximum supination and
10-second ON duty, and 7-second OFF duty cycle then applying the elbow extension unit. Velcro is
commonly is selected to produce rhythmic muscle used to prevent slippage of the orthosis during
contraction. The stimulus amplitude threshold is pronation. With this technique, the authors
determined by increasing the amplitude of the achieve maximum mechanical leverage on the
stimulus until muscles start to contract. The am- pronator teres muscle and strengthen the ability of
plitude then is reduced gradually until no con- biceps to supinate the forearm.
tractions are apparent. This amplitude threshold
then is doubled and used routinely. If necessary,
Neuromuscular electrical stimulation
the amplitude is decreased to make contractions
and dynamic bracing protocol
tolerable.
The home-based program consists of approx-
imately 1 hour per day of electrical stimulation
Dynamic orthotic traction
preceded by dynamic bracing. Treatment time can
The orthotic device has two parts: a wrist/hand be divided into two 30-minute sessions or three
unit and an elbow unit. The wrist/hand unit 20-minute sessions, depending on the working
consists of a thermoplastic rigid support that schedules of the parents and availability of care-
positions the wrist in extension (Fig. 1). This unit givers. After the parents and caregivers are
also has an adjustable finger platform that locks the instructed, children are fitted with the wrist and
proximal interphalangeal (PIP) and distal inter- elbow unit. Adhesive, reusable electrodes then are
placed on the bellies of the wrist and finger
extensor muscles. Once the amplitude is deter-
mined, the extensor muscles are stimulated for
10 seconds to obtain a tetanic contraction, fol-
lowed by 7 seconds with no electrical stimulation.
During the period with no stimulation, the patient
is told to flex the fingers repeatedly so that they
learn which muscles to contract to flex the fingers
and prevent co-contraction. At the beginning of
the treatment, the rubber bands in the outrigger
part of the dynamic brace are strong enough to
extend the fingers, and then, as the extensor
tendons become stronger, the strength of the
rubber bands is decreased gradually. After each
session, children are allowed to continue with
their daily activities. The orthotic devices and the
Fig. 1. Placement of electrodes to the extensor surface electrode positioning are reviewed every time the
of the forearm together with application of the dynamic patient is seen in the clinic, initially 2 weeks after
brace. starting treatment and then every month, to
604 L.R. Scheker, K. Ozer / Hand Clin 19 (2003) 601–606

assure that the orthosis and the stimulation are


being applied correctly. At night, the finger plat-
form is connected to a dorsal outrigger using a
static Velcro strap to resist the contracted extrin-
sic flexors and maintain the ROM gained during
the day.
The children are assessed using the Zancolli
classification at the start of treatment and every
month thereafter (Table 1) [32]. Patients who
comply with the program show a significant
improvement in quality of movement of the upper
extremity. They are able to extend the wrist and
fingers leading to better control and use of the
hand. Fourteen out of 19 patients moved two Fig. 3. Four months’ follow-up of the same patient
grades in Zancolli’s classification (Figs. 2 and 3). reveals well controlled wrist and finger motion.
None of the patients have reported any pain or
injury related to the stimulation. Eight patients
reported some discomfort while wearing the splint ued, showed signs of regression of spasticity and
because it did not fit perfectly. Reforming the had to begin the program again.
thermoplastic arm support relieved this discom- The results of the current protocol showed
fort. In all patients co-contractions were reduced. that the use of NMES with dynamic orthotic trac-
One patient, in whom treatment was discontin- tion during the day and static orthosis at night
is a quick and dramatically effective method to
improve function in children with spasticity of the
upper extremity caused by cerebral palsy. The
efficacy of this treatment was confirmed by the
improvement observed in the Zancolli classifica-
tion scoring. All patients improved at least one
grade during the treatment period, with progress
noted as early as 4–6 weeks following the
application of the protocol.
Previous reports on the use of electrical stim-
ulation for the management of spastic upper ex-
tremities have reported conflicting results [22,24,
26]. Atwater first reported two patients, aged 11
and 12 years, who received stimulation of their
wrist extensors [22]. The protocol consisted of
three 20-minute treatment sessions each day for 8
weeks. Those investigators reported no improve-
ment at the end of the treatment period. Later,
Carmick used a similar protocol using 15 minutes
of stimulation with a fixed 300-microsecond pulse
width and a static wrist extension splint [23,24].
The electrodes were applied to the nonspastic
wrist extensors and the spastic flexors at the same
time. Electrical stimulation, although applied to
the spastic flexors, did not increase the spasticity,
and improved overall function. Finally, Wright
and Granat reported the results of eight patients
receiving FES to wrist extensors [26]. The pro-
Fig. 2. At the beginning of the combined protocol of tocol consisted of 30-minute daily sessions of
NMES and dynamic bracing, the patient is unable to cyclic FES (300 microsecond) for 6 weeks.
extend the right wrist without the help of the contralat- Significant improvement of hand function was
eral side. observed at the end of the study. These studies
L.R. Scheker, K. Ozer / Hand Clin 19 (2003) 601–606 605

support the usefulness of electrical stimulation. Copiah County. Miss Dev Med Child Neurol 1984;
The protocol used in the current study differs 26:195–9.
from these reports in several ways. First, the use [2] Evans P, Elliott M, Alberman E, Evans S. Prev-
of NMES is aimed at nonspastic but weak alence and disabilities in 4 to 8 year olds with cere-
bral palsy. Arch Dis Child 1985;60:940–5.
extensor compartment muscles. This approach
[3] Murphy CC, Yeargin-Allsopp M, Decoufle P,
provides effective stretching and elongation of the Drews CD. Prevalence of cerebral palsy among
spastic flexors while strengthening the weak ten-year-old children in metropolitan Atlanta, 1985
extensor muscles. Furthermore, the use of the through 1987. J Pediatr 1993;123:S9–13.
dynamic orthotic device simply reinforces the [4] Manske PR. Cerebral palsy of the upper extremity.
intrinsic muscles, providing better fine motor Hand Clin 1990;6:697–709.
movement and control. [5] Aicardi J, Bax M. Cerebral palsy. In: Aicardi J,
Certain elements of the current protocol need editor. Diseases of the nervous system in childhood.
further investigation. The authors noted that the 2nd edition. London: Cambridge University Press;
effects of therapy decrease after discontinuation 1998. p. 210–40.
[6] Goldner JL. Surgical reconstruction of the upper
of therapy. The duration of effectiveness of the
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therefore recommend a lifelong application of the [7] Palmer FB, Shapiro BK, Wachtel RC, Allen MC,
protocol, but after the first 12 months it is possible Hiller JE, Harryman SE, et al. The effects of
to reduce the frequency of the NMES and the physical therapy on cerebral palsy: a controlled trial
period of use of dynamic bracing. Patients who in infants with spastic diplegia. N Engl J Med
were classified as Zancolli I after treatment 1988;318(13):803–8.
continued a maintenance program consisting of [8] Bleck EE. Cerebral palsy. In: Bleck EE, Nagel DA,
NMES and dynamic traction once a week for editors. Physically handicapped children: a medical
30 minutes. They also were instructed to wear the atlas for teachers. 2nd edition. New York: Grune
Stratton; 1982. p. 311–9.
brace at night. These patients were monitored
[9] Fisk JR, Supan TJ. Cerebral palsy. In: Goldberg B,
periodically by their local therapist and reviewed Hsu JD, editors. Atlas of orthoses and assistive
every 6 months. Many of the authors’ patients devices. 3rd edition. London: Mosby; 1997.
who have been on the maintenance program for p. 533–42.
more than 2 years use the electrical stimulator on [10] Molnar GE. Orthotic management of children. In:
alternate days, often using the brace only at night. Redford JB, editor. Orthotics et cetera. 2nd edition.
The first patient treated has been on the mainte- London: Williams and Wilkins; 1986. p. 352–87.
nance program for 5 years, 5 months and has not [11] Hoffer MM, Garrett A, Koffman M, Guilford A,
shown any signs of regression of spasticity. With Noble R, Rodon G. New concepts in orthotics for
this protocol, the authors have noted a decrease in cerebral palsy. Clin Orthop 1974;102:100–7.
[12] Joynt RL, Leonard JA. Dantrolene sodium suspen-
spasticity of the flexor pronator muscles and an
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The mechanism of action of NMES and theoretical and practical considerations. Can J
dynamic traction is not yet known. One may Neurol Sci 1987;14:510–2.
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NMES and dynamic bracing, we stimulate a nat- baclofen for spasticity in cerebral palsy. JAMA
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alternative for the management of spasticity.
[16] Calderon-Gonzalez R, Calderon-Sepulveda R,
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combination of both. [17] Koman LA, Mooney JF, Smith B, Goodman A,
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Neurol 1997;39:185–93. 226–32.
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Hand Clin 19 (2003) 607

Part 2: surgical management


Introduction
Michael A. Tonkin, MD, FRACS
Department of Hand and Peripheral Nerve Surgery, Royal North Shore Hospital,
St. Leonards, NSW 2065, Australia

This section is appropriately introduced by the section on thumb deformity comes from my
Eduardo Zancolli, for the principles he has own experience.
espoused have guided all those who have fol- The outcome paper of Johnstone and col-
lowed. The repetition of these principles by other leagues is a welcome summary of methods of
authors is a testament to his influence. assessment of surgical results in cerebral palsy,
Little is written about the management of sometimes a most difficult objective exercise.
elbow deformity in cerebral palsy, and even less Finally, the work of Bertelli and colleagues de-
has been directed toward the shoulder. It is for scribes encouraging results from neurosurgical
this reason that Landi’s contribution has been intervention, procedures that may be less familiar
included in this section, although much of the than the more common orthopedic procedures
work is devoted to spasticity as a consequence of of muscle releases, tendon transfers, and joint
head injury and stroke. However, many of the fusions. The result is a comprehensive compilation
principles remain the same. Gschwind and Van of surgical indications, techniques, and results in
Heest discuss the management ‘of pronation the management of cerebral palsy in the upper
deformity and wrist and finger deformity, and limb.

E-mail address: mtonkin@surgery.usyd.edu.au

0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00096-9
Hand Clin 19 (2003) 609–629

Surgical management of the hand in infantile


spastic hemiplegia
Eduardo A. Zancolli, MD*
National Academy of Medicine, Orthopaedics and Traumatology,
3092 Las Heras Avenue, Buenos Aires, Argentina

The spastic hand in cerebral palsy represents General preoperative evaluations in cerebral palsy
one of the most complex problems in upper limb
The principal evaluations for selecting cerebral
reconstructive surgery. The pathology depends on
palsy patients suitable for hand surgery refer to
lesions of the central nervous system, resulting in
etiology, general neurologic condition, type of
severe motor imbalance and sensory impairment
neuromuscular disorder, extent of limb involve-
of the hand. Despite these difficulties, upper
ment (hemiplegia, tetraplegia, diplegia, triplegia, or
extremity reconstructive surgery has become an
monoplegia), age, sensory impairment, and upper
excellent aid in the management of selected cases
limb deformity (wrist and finger classification).
of the spastic hand when properly indicated and
performed, as shown by Goldner 1955 [1], 1975
[2], Swanson 1968 [3], Zancolli 1968 [4], 1975 [5], Etiology
and 1979 [6]. Lesions of the intercranial central nervous
Cerebral palsy may be defined as any disorder system may have their origins in pre-, peri-, and
of neuromotor functions secondary to cerebral postnatal periods. In the author’s experience,
damage occurring prenatally, at birth, or early in patients with perinatal brain damage are best
the postnatal period. suited to peripheral reconstructive surgery when
The purpose of this article is to describe the the patient is a spastic young hemiplegic.
surgical rehabilitation of the spastic upper limb in Perinatal injuries have two main causes,
infantile hemiplegia, in which peripheral recon- trauma and anoxia during the time of birth.
structive surgery gives the best results. The Blumel et al [7] found the most frequent lesions
author’s experience is based on a series of 113 were caused by trauma at birth (13%), anoxia
upper limb spastic cases operated on over a period (24%), prematurity (32%), congenital defects
of 33 years. The final results refer to 47 patients (11%), and postnatal damage (7%).
evaluated from a group of 91 patients (23% were
hemiplegics). Of these cases, 84% were treated in Neurologic lesions
the Rehabilitation Center of Buenos Aires (Ar-
gentina). Satisfactory results were obtained in Severe neurologic disorders are usually absent
92% of the most favorable cases, groups I and II in the typical infantile spastic hemiplegia, the
of the author’s classification. condition most adapted to peripheral reconstruc-
Careful examination, testing, and evaluation tive surgery of the upper limb. Reconstructive
by the neurologist, the surgeon, and the occupa- surgery is contraindicated in severe neurologic
tional therapist are of major importance in the alterations, such as defects in speech, vision, and
selection of patients for surgical rehabilitation. hearing, and mental retardation, which are
generally present in spastic tetraplegia and extra-
* Correspondence. Avenida Alvear 1535, 1014 Bue- pyramidal neuromuscular disorders. Mild alter-
nos Aires, Argentina. ation of the mental condition in hemiplegic cases
E-mail address: adriananegri@hotmail.com is not a surgical contraindication.
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00034-9
610 E.A. Zancolli / Hand Clin 19 (2003) 609–629

Type of neuromuscular disorder 2. Hypertonicity increases when the affected


muscles are gradually stretched passively.
Patients with neuromuscular disorders can be
3. Hypertonicity is constant except during sleep
classified into three types: spastic or pyramidal,
or under anesthesia and it does not diminish
extrapyramidal, and mixed. Determining the type
with orthoses or manipulations.
of neurologic disorder is of paramount impor-
4. Hypertonicity produces what has been named
tance in selection of candidates for reconstructive
synchronous activity or co-contraction [8]. Its
surgery (Fig. 1).
clinical manifestation is represented by an
abnormal reaction of the spastic muscles
Pyramidal type (hypertonicity)
during rest or when the muscles are acting
In this type of neuromuscular disorder, the
as antagonists. Antagonist spastic muscles
most frequent type of neuromuscular disorder in
remain electrically active on flexion and
cerebral palsy (70%), the classic deformity of the
extension of the involved part of the limb. A
upper limb is characterized by a combination of
typical finding of this phenomenon is the
spastic and paretic or flaccid paralysis. It depends
persistent activity of the flexor muscles of the
on the damage of the corticospinal tracts. The
wrist when complete finger and wrist exten-
principal clinical characteristics of spasticity in the
sion are attempted, especially at the level of
infantile hemiplegic patient are:
the flexor carpi ulnaris (FCU). Synchronous
1. Flexor–pronator deformity of the upper limb activity alters the normal grasping and release
(Fig. 1A). This deformity depends on the patterns of the hand. Co-contraction can be
spasticity of the extrinsic muscles of the a favorable situation in some tendinous
forearm and hand represented by the flexor– reconstructive procedures [3]. Co-contraction
pronator muscular mass. Flaccid paralysis or thus favors active extension of the wrist after
paresis of the opposite muscles (extensor– the transfer of a spastic FCU tendon to the
supinator mass) is present. extensors of the wrist. Under this condition,

Fig. 1. Most frequent types of neuromuscular disorders. (A) Spastic. (B,C) Athetosis. In athetosis, the fingers show
opposite postures in the same hand, which is a typical clinical manifestation of extrapyramidal disorders (see Fig. 17).
E.A. Zancolli / Hand Clin 19 (2003) 609–629 611

the co-contraction of the FCU produces by efforts to move or by any emotional or


wrist extension caused by its synchronous environmental stimuli. Typically the patient
activity (Green’s procedure [9]). may show different asymmetric finger deform-
5. Overactive stretch reflex is one of the most ities in the same hand (Fig. 1B and C). Abn-
typical manifestations in the diagnosis of ormal hand movements and deformities
hypertonicity. It is produced by failure of the usually decrease if the patient is distracted.
normal muscle lengthening reaction to elon- 2. Delayed postural development. The disorder
gation by sudden passive stretching. Owing to is usually generalized, but unilateral involve-
the hyperactive stretch reflex, the spastic ment also occurs.
muscle contracts at the same point in the arc 3. Hypotonus in infancy. This condition may
of motion each time a passive elongation change with growth to increased tonus in
maneuver is produced. The overactive stretch stress situations.
flex is absent in extrapyramidal neuromuscu- 4. Decreased reflexes in infancy that may
lar disorders but present in most of the mixed become hyperactive later.
group, in which spasticity is predominant over 5. No tendency to muscular contracture.
athetosis. The stretch reflex should be in- 6. Absence of Hoffman reflex and stretch reflex.
vestigated for each muscle or group of 7. Frequently tactile gnosis of the hand is
muscles. Spasticity and overactive reflexes preserved.
can be decreased by certain surgical proce-
Soft tissue procedures performed in the pure
dures, such as lengthening of distal tendons or
athetoid patient are contraindicated because they
release of proximal muscle insertions.
may produce a new undesirable opposing de-
6. Myostatic contracture. This represents a sec-
formity that may be totally uncontrollable by the
ondary muscular fibrosis after a long-standing
patient. In this situation a transference of the
hypertonicity. This fibrosis is seen frequently
initial distorted position to other muscle groups is
in the flexor muscles of the wrist and digits. It
usually produced. Soft tissue procedures also are
persists with the patient under general anes-
contraindicated in other extrapyramidal muscular
thesia or peripheral nerve blocking. Muscular
disorders, such as chorea, ataxia, tremor, and
fibrotic retraction is not a contraindication to
rigidity. Clonus is a poor prognostic sign for the
surgical reconstruction of the hand. It is ob-
success of tendon transfers. In athetosis it is
served frequently after infancy and during or
possible to consider surgical procedures of bones
after adolescence. It can be reduced preoper-
(osteotomy) or joints (fusion).
atively by physical methods of stretching with
Soft tissue procedures also are absolutely
casts or braces. During surgery it can be corrected
contraindicated in a young child with extrapyr-
by tenotomy preserving muscular continuity.
amidal hypotonus, which may change with growth
7. Hypertonicity increases with emotional stim-
to abnormal athetoid involuntary movements and
uli. If this reaction is a predominant clinical
increased tonus under emotional stimuli. [6].
finding it is difficult to obtain a reasonable sur-
gical result even if the hand deformity is mild.
Mixed type
In these cases the patient is incapable of col-
The mixed type of neuromuscular disorder is
laboration in the postoperative reeducation [6].
a common form. Most frequently spasticity is
Extrapyramidal type associated with athetosis and is characterized by
The extrapyramidal type of neuromuscular a combination of hypertonus and reduced abnor-
disorder is represented by athetosis, ataxia, mal movements (Fig. 1). Reconstructive surgery
tremor, or rigidity. The most frequent extrapyra- through soft tissue techniques may be indicated in
midal disorder is athetosis—approximately 25%, the mixed type of neuromuscular disorder if
usually produced by neonatal anoxia and eryth- hypertonicity and, consequently, overactive stretch
roblastosis fetalis. Athetosis is the result of reflexes are present and dominant.
dysfunction of the basal ganglia. It is character-
ized by the following clinical findings: Age
1. Abnormal, involuntary, and poorly coordi- Surgical correction of spastic upper limb
nated movements with varying degrees of deformities usually is indicated after 6–7 years of
tension. The deformity decreases at rest and age. Maturation of the central nervous system has
disappears during sleep, but it is accentuated occurred by this age, and the child is usually
612 E.A. Zancolli / Hand Clin 19 (2003) 609–629

cooperative during postoperative rehabilitation. wrist and finger flexion contracture (occasionally
After adolescence and adulthood, patients are accompanied by flexible swan-neck deformity)
not generally good candidates for reconstructive and thumb deformity. The flexed wrist usually
surgery, because they usually have accepted their tends to deviate ulnarly. Two types of thumb
cosmetic and functional problems. deformities may be present: (1) adduction con-
tracture (adduction of the first metacarpal) and
Hand sensory impairment (2) flexion–adduction contracture (thumb-in-palm
deformity) (Fig. 2). All these deformities are
Based on the author’s experience [10], all
variable in severity and reducibility by external
patients with a pure spastic neuromuscular
passive maneuvers of thumb extension combined
disorder have some deficit of sensibility in pro-
with wrist flexion.
prioception (conscious control of position, mo-
tion, and power) and stereognosis (ability to
Hand deformity classification: based in voluntary
recognize objects by touch only). In the classic
grasp and release patterns between the wrist
infantile hemiplegia with a pure spastic neuro-
and fingers
muscular disorder, the affected hand preserves the
ability to recognize the physical characteristics of Since the author’s publications in 1968 [4],
objects (shape, size, texture) or protective sensa- 1979 [6], 1981 [12], and 1987 [10], the spastic
tions (hot, cold, pain, pressure), but the patient is infantile hand has been classified into three
unable to name the objects because of the cortical groups, based on the voluntary possibility of the
involvement. This is the typical condition in patient to produce release of the fingers through
spastic patients. Defects in stereognosis are not active wrist flexion (Fig. 3). The surgical program
a contraindication for attempting to improve
hand motor function and appearance by surgery
[11]. Only in patients in whom spasticity is
combined with some athetoid component may
hand sensibility be preserved completely.
Sensibility tests used in these patients are: (1)
pinprick test (protective sensation), (2) recogni-
tion of size and shape of objects (differentiation of
cubes from marbles in small children), (3)
Seddon’s coin test (tactile gnosis), which is the
author’s preferred investigation for sensibility
evaluation, (4) two-point discrimination (Mo-
berg’s paper clip test, tactile gnosis), and (5)
position sense (proprioception or body position
movement recognition).
It is characteristic that the spastic hemiplegic
child with stereognosis defects prefers to use the
normal hand. He or she uses the affected hand
only when necessary for bimanual activities and
always as an assistant limb. When sensibility is
not affected, as exceptionally may occur in mixed
groups, better function can be obtained and
independent function of the affected hand can be
expected after reconstructive surgery.

Upper limb deformity (pronator–flexor spastic


contracture)
The typical flexion–pronation deformity of the
spastic upper limb in cerebral palsy is character-
ized by the following components: (1) elbow
flexion contracture, (2) forearm pronation con- Fig. 2. Typical spastic deformities of the thumb. (A)
tracture, and (3) hand deformity, characterized by Adduction. (B) Thumb-in-palm (flexor–adduction).
E.A. Zancolli / Hand Clin 19 (2003) 609–629 613

of the wrist and finger deformities is closely (see Fig. 3III, Fig. 8). Synergism between the wrist
related to this classification. muscles and finger muscles is completely lost. This
condition is the most complex to improve by
Group I reconstructive surgery. Partial release of all the
In this group of patients, flexion spasticity is spastic muscles of the upper limb is the author’s
minimal. The patient can extend completely the choice in this group of patients.
fingers in a neutral position of the wrist or with less All groups usually are associated with thumb
than approximately 20 –30 of active wrist flexion. deformity—either in adduction or flexion-in-palm
Here the principal spasticity is localized at the (see Fig. 2)—elbow flexion contracture, and pro-
FCU muscle. The principal deficits in this group of nation contracture of the forearm of variable
patients are the lack of complete active wrist severity. The degree of sensory impairment varies
dorsiflexion when the fingers are totally extended between groups.
and the presence of thumb deformity [11] (Figs. 3I Swan-neck deformity may be present in all
and 4). The general appearance of the upper limb is groups but particularly in group II. This de-
satisfactory and the emotional influence on spas- formity reduces substantially after the flexion
ticity is usually mild or almost absent. Pronation contracture of the wrist is surgically corrected
spasticity of the forearm may be present. (Fig. 9).

Group II
In these patients, the fingers also can be Surgical goals
actively extended, as in group 1, but only with
The principal goals in surgical reconstruction
more than 20 –30 of active wrist flexion (see Fig.
of the spastic hand are directed at improving
3II). Principal spasticity is localized at the wrist
grasp and release patterns between the wrist and
and finger flexors and thumb. In severe cases in
digits, hand appearance, function of the affected
group II, the wrist needs to flex completely to
hand, and the psychologic status of the patient
permit complete or partial finger extension.
and the family.
Group II has two subgroups according to the
functional condition of the extensor muscles of
the wrist (IIA and IIB). In subgroup IIA the
Prognosis
patient can actively extend, partially or totally, the
wrist, when the fingers are flexed (Figs. 5 and 17). According to the classification previously de-
This means that the extensor muscles of the wrist scribed, basic grasp and release functions of the
and fingers are active and voluntarily controlled hand can be significantly improved in groups I
and that the main spasticity is localized in the and II. In these cases it is possible to obtain
flexor muscles of the wrist and fingers. In these improved finger release with decreased flexion of
cases, obviously, it is unnecessary to perform the wrist, decreased flexion contracture of the
tendon transfers to extend the wrist. In subgroup elbow, wrist, and fingers, decreased forearm
IIB the patient cannot actively extend the wrist pronation contracture, and correction of thumb
with the fingers flexed because of the flaccid deformity to improve gripping and lateral pinch.
paralysis of the wrist extensor muscles or because The main concept in surgical reconstruction of
the wrist flexor muscles are severely contracted the spastic hand is that an improvement in the
(Figs. 6 and 16). Tendon transfers to extend the existing pattern of function can be achieved by
wrist are necessary in cases of subgroup IIB when improving the balance between the spastic pro-
the wrist extensor are paralyzed. Occasionally the nator–flexor muscles and the normal or paretic
wrist flexors are not affected by spasticity or extensor–supinator muscles. Attempts to modify
myostatic contracture (Fig. 7). radically the preoperative existing patterns of
hand activity or overcorrection usually lead to
Group III poor results. Only in mild spasticity (group I) is it
Here the spasticity of the hand is severe and possible to obtain normal complete and simulta-
localized at the flexor–pronator mass, as in the neous extension of the wrist and fingers through
other groups. The extensor muscles of the wrist release of the FCU.
and fingers are totally paralyzed. The patient In group II, reconstructive surgery is related
cannot actively extend the fingers, even with to the condition of the flexor–pronator and
maximal active or passive flexion of the wrist extensor–supinator muscles.
614 E.A. Zancolli / Hand Clin 19 (2003) 609–629

Fig. 3. Grouping of wrist-fingers deformities in the spastic infantile hand. Group I, group II with subgroup A and B, and
group III.
E.A. Zancolli / Hand Clin 19 (2003) 609–629 615

Fig. 3 (continued )

Fig. 4. Group I of spastic hand. The fingers can only


In group III, surgery is indicated exclusively to extend completely with a wrist flexion spasticity of 30 .
improve appearance, hygiene, and comfort. This
can be achieved by reducing the spasticity of the
flexor–pronator muscles without pretending to
obtain voluntary hand grasp and release.

Fig. 5. (A) Group IIA of an infantile spastic hemiplegic hand. The fingers can voluntary flex with the wrist in 65 of
flexion. (B) The fingers extend with the wrist in 45 of flexion. (C) With the fingers flexed, the wrist extends voluntary up
to 25 of flexion. Spastic contracture of the wrist flexors prevents complete wrist extension.
616 E.A. Zancolli / Hand Clin 19 (2003) 609–629

Fig. 6. (A) Group IIB in an infantile hemiplegic patient. The wrist cannot voluntary extend with the fingers flexed. The
wrist extensor muscles are paralyzed. (B) The fingers can only extend with the wrist in 90 of wrist flexion.

Surgical indications 6. Basic sensibility present, even with some


impairment of proprioception and tactile
According to the preoperative conditions, the
gnosis.
best candidates for peripheral reconstructive
7. Voluntary control of the spastic muscles and
surgery are:
voluntary ability to open the fingers in flexion
1. Spastic type of neuromuscular disorder. (preserved synergism) (groups I and II).
Surgical reconstruction of the upper limb
It is important for the patient to have some
through soft tissues procedures, such as
voluntary control of the spastic muscles and some
tenotomies and tendon transfers, is indicated
voluntary ability to release and close the fingers
in pure spastic hands but also in mixed cases
for satisfactory results to be achieved through
in which mild athetosis is combined (mixed
reconstructive surgery. Experience has shown the
group).
author that the most favorable cases for surgery
2. Sufficient mental condition and emotional
are those in which it is possible to open the fingers
stability (usually present in spastic hemi-
by active flexion of the wrist. For this reason wrist
plegia).
arthrodesis should not be a primary option in
3. Low emotional influence on spasticity.
typical spastic infantile hemiplegia if the patient
4. Infantile hemiplegia (especially perinatal).
has the ability to extend the fingers through wrist
5. Young patients (infantile or adolescent),
flexion. Synergism between wrist and fingers
ideally after 6–7 years of age. Training is
should be preserved whenever possible.
more effective after this age.

Fig. 7. A severe case of infantile spastic hand. (A) The fingers open with only 110 of voluntary wrist flexion. (B) The
wrist only extends passively. This means that the wrist extensor muscles are paralyzed and its flexor muscles are not
spastic or with myostatic contracture. Spasticity is only present in the long flexor muscles of the fingers.
E.A. Zancolli / Hand Clin 19 (2003) 609–629 617

tion for peripheral surgical reconstructive


procedures. The surgical program depends
on the severity and type of deformity at the
different levels of the upper limb. The most
common deformities corrected by reconstruc-
tive surgery are: thumb deformities, flexion
contracture of the wrist and fingers, flexion
contracture of the elbow, and pronation
contracture of the forearm. Seldom, swan-
neck deformities of the fingers need to be
corrected. Surgery of the thumb is indicated
to hold the digit out of the palm during grasp.
The surgical procedures indicated depend on
Fig. 8. An infantile hemiplegic spastic patient with
the type of deformity present: adduction or
a group III of wrist-fingers deformity. The patient
adduction–flexion contractures (thumb-in-
cannot voluntarily open the hand. Typical flexor–
pronator deformity. palm deformity) (see Fig. 2).

Flexion contracture of the elbow is common


The author only considers wrist fusion in in the spastic upper limb and frequently is in-
atypical spastic cases, such as the residual fluenced by emotional stimuli. Correction of
deformities of failed previous operations in which marked flexion contracture of the elbow improves
tendon transfers or other soft tissue procedures significantly the appearance and function of the
are impossible to perform or are contraindicated, hand.
and in some extrapyramidal neuromuscular dis- Pronation contracture of the forearm is cor-
orders [4]. rected when severe and when the patient has
difficulty carrying out activities of daily living
8. Capacity to concentrate and cooperate during
because of lack of supination. A mildly pronated
the postoperative period.
forearm is a useful position for hand function and
9. Good motivation of the patient and family
its correction is not essential.
support.
Flexion contracture of the wrist and fingers is
10. Adequate behavioral patterns.
corrected through surgical techniques based on
11. Good general neurologic condition, which is
the classification presented (groups I, II, and III).
generally present in infantile spastic hemi-
This indicates that during the preoperative
plegia.
examination it is of great importance to note
12. Spastic flexion–pronation deformity of the
the degree of wrist flexion needed to enable the
upper limb. This is the most suitable condi-
patient to extend the fingers completely or
partially. It is also important to determine if the
wrist can be extended with the fingers flexed
(subgroups IIA or IIB). This test demonstrates
the degree of spasticity of the flexor muscles of
the wrist and fingers, the functional condition of
the wrist extensors, and the degree of voluntary
control. This information is needed to determine
the most appropriate surgical program for each
patient.
It is of paramount importance that all the
existing deformities of the upper limb (elbow,
forearm, and hand) should be corrected at the
same surgical procedure. A contracted thumb
thus must be simultaneously corrected with the
Fig. 9. Group IIB of spastic hand deformity with rest of the hand to allow better active extension of
pronounced swan-neck deformities of the fingers when the fingers and lateral pinch grip.
the wrist flexes. Recurvatum of the proximal interpha- Severe swan-neck deformities are usually cor-
langeal joints reduces if the wrist is extended passively. rected in a secondary surgical stage. Severe spastic
618 E.A. Zancolli / Hand Clin 19 (2003) 609–629

equinus foot preferably should be corrected


before hand surgery with the aim to eliminate
foot hypertonicity that helps with postoperative
rehabilitation of the hand.

Surgical procedures
Surgical procedures most frequently indicated
are: muscle release, tenotomy, fasciotomy, tendon
lengthening, tendon transfer, capsuloplasty, te-
nodesis, arthrodesis, osteotomy, and neurotomies.
The selection of the procedure basically depends
on the type and severity of the deformity, the type
of neuromuscular disorder, the functional defi-
ciencies, the age of the patient, and the surgeon’s
preferences.
The operative techniques are described in
relation to the different deformities.

1-Flexion contracture of the wrist and fingers


This deformity basically results from the
spasticity and occasional myostatic contracture
of the flexor muscles of the wrist and fingers. The Fig. 10. Most frequent indication in the wrist in mild
goal of surgery is to improve the active release of cases of spastic hand group I. The FCU muscle is
the hand without affecting the voluntary closure lengthened with tenotomy preserving the muscular
of the digits and grasping functions. This is fibers. This procedure improves simultaneous wrist and
achieved by preserving the balance between finger extension.
the flexor and extensor muscles of the wrist and
digits.
According to the grouping of hand deform- The treatment of the thumb deformity and
ities, the author’s surgical program to obtain the occasional elbow flexion spasticity is described in
desirable balance between the wrist and finger subgroup IIB.
muscles is as follows.
Group II
Surgical technique in group II of flexion
Group I
contracture of the fingers and wrist depends on
The principal muscular co-contraction in these
severity of spasticity and functional condition of
cases basically is located in the FCU. Our aim is
the wrist extensor muscles (subgroups IIA and IIB).
to lengthen the FCU through a simple tenotomy,
Incisions used in these cases are shown in Fig. 11.
preserving muscle continuity. In this type of
The desired correction of wrist and fingers
tenotomy, the tendinous part is sectioned, pre-
spasticity of this group of patients is obtained by
serving the muscular fibers. After the release
the author’s technique, described in 1968 [4] and
a passive stretching maneuver permits lengthening
1979 [6], of medial epicondyle aponeurotic release,
of the muscular unit. Tenotomy is located
tenotomies, and tendon transfers.
proximal to the distal end of the muscular belly
(Fig. 10). Aponeurotic release of the medial epicondyle
If the wrist and finger flexor muscles and the muscles. This is an effective technique in patients
pronator teres are spastic, an aponeurotic release with mild and median spasticity of wrist and
of the medial epicondyle muscles is added. This finger flexor muscles. In this procedure spasticity
release is located 5 cm distally to the medial is reduced because all the deep attachments of the
epicondyle. This technique is sufficient to release medial epicondyle muscles are released at the
flexion contractures of the wrist and fingers in this proximal third of the forearm. The muscular
group of patients. It is described in group II of the fibers are left intact. Mild and median spastic
spastic flexion–pronation deformity. patients are those in whom preoperative voluntary
E.A. Zancolli / Hand Clin 19 (2003) 609–629 619

Fig. 11. (A) Incisions in cases of group IIB. 1, biceps release; 2, medial epicondyle aponeurotomy; 3, pronator teres
approach to release or transfer of the pronator teres; 4, FCU approach (transference to extensor carpi radialis brevis
through the interosseous membrane); 5, adductor pollicis release; 6, radial approach for metacarpal-sesamoid synostosis
(see Fig. 14). (B) Dorsal approach in the wrist to reach the extensor tendons of the fingers and thumb.

finger extension is obtained with less than muscle continuity) of some retracted muscles
approximately 70 of active wrist flexion. (flexor digitorum superficialis and profundus and
In the aponeurotic release of the medial flexor carpi radialis). This complementary release
epicondyle muscles, a transverse excision of the is located distal to the proximal aponeurotic
superficial fascia is made around the whole release, at approximately the level of the muscu-
muscular mass. This is followed by a complete lotendinous junction in the middle of the forearm
excision of the septa that separate the muscles. [12]. Myostatic contracture is recognized under
Each septum is released deeply where one can see anesthesia in which a passive extension maneuver
the median and ulnar nerves covered by the is unable to obtain complete and simultaneous
muscles. The muscular bellies are left intact, extension of the wrist and fingers.
particularly the FCU if this muscle is to be The author does not indicate the classic
transferred to the extensor tendons of the wrist muscular slide technique as described by Page
(subgroup IIB) (Figs. 12A, 13A). [13]; see also Inglis and Cooper [14] and White
In cases of severe spasticity or myostatic [15]. Using this technique it is difficult to calculate
contracture of the wrist and fingers muscles, in the necessary degree of muscular release and
which finger extension is only possible with consequently overcorrection could be the result,
greater than 70 of wrist flexion, one adds tendon causing the fingers to lose flexion and grasp
releases through selective tenotomies (preserving abilities or to produce an intrinsic plus deformity.
620 E.A. Zancolli / Hand Clin 19 (2003) 609–629

Fig. 12. (A) Most frequent surgical program indicated in subgroup IIB of a spastic hemiplegic hand with a thumb-in-
palm deformity, mild flexion contracture of the elbow, and pronation contracture of the forearm. 1, Biceps lengthening
with its muscle fibers in continuity; 2 and 3, excision of part of the superficial fascia and all septae of the medial
epicondyle muscles; 4, pronator teres release (pronator quadratus remains active); 5, transference of the FCU through an
ample window in the interosseous membrane to the extensor carpi radialis brevis tendon; 6, lengthening in continuity of
the flexor pollicis longus tendon. It is necessary to avoid too much relaxation; 7, release of the abductor pollicis longus at
its proximal end. When the metacarpophalangeal joint of the thumb is lax and deforms in hyperextension, a sesamoid-
metacarpal fusion is indicated during the same surgical stage (see Fig. 14). (B) 1, Transference of FCU to extensor carpi
radialis brevis with partial excision of the extensor retinaculum; 2, transference of brachioradialis to abductor pollicis
longus; 3, transference of palmaris longus to extensor pollicis longus through the interosseous membrane (too much
tension should be avoided). Balance of tension needs to be maintained between the lengthened flexor pollicis longus and
the transferred extensor pollicis longus. In cases with a single adduction contracture of the thumb, the flexor pollicis
longus is left unreleased and the extensor pollicis longus is translocated radially. (C) Transference of the pronator teres
through the interosseous membrane to produce forearm supination. The tendon is fixed to the radius.

Another possible complication of an excessive flexors—flexor carpi radialis and palmaris


muscular slide procedure is the loss of vascular- longus—must remain in place to avoid an un-
ization of the medial epicondyle muscular mass. desirable permanent wrist hyperextension post-
operatively. The author prefers to pass the FCU
through an ample window in the interosseous
Tendon transfer of the FCU to the extensor carpi
membrane—proximal to the pronator quad-
radialis brevis. This technique is indicated only
ratus—and suture it to the extensor carpi radialis
when active wrist extensors are weak or completely
brevis tendon (see Figs. 12A,B, 13B,C). The
paralyzed (subgroup IIB) (see Figs. 12A,B). It is
tension of the transfer is calculated during surgery.
not indicated, obviously, in subgroup IIA, in
It is considered correct when the wrist maintains
which active wrist extension is present (see Fig.
20 of extension under the effect of gravity.
5). In the classic Green’s procedure [9,16], the FCU
tendon is passed around the ulnar border of the
wrist and is fixed to the extensor carpi radialis Group III
brevis tendon, because it is the major dorsiflexor of In this group there is a permanent and severe
the hand. The goal is to obtain wrist extension and flexion contracture of the wrist and fingers,
supination of the forearm. The other wrist particularly under emotional stimuli, and it is
E.A. Zancolli / Hand Clin 19 (2003) 609–629 621

Fig. 13. (A) Excision of part of the superficialis fascia and all the septae of the medial epicondyle muscles. This gives
a partial release of spasticity of the muscles inserted in the medial epicondyle tuberosity. (B) Transference of the FCU
tendon (1) through an ample window opened in the interosseous membrane (2) proximally to the pronator quadratus (3).
(C) FCU (1) and the brachioradialis (2) tendons to activate the extensor carpi radialis brevis and abductor pollicis longus
(3), respectively.

impossible to voluntarily open the hand. The are then extended to a median flexed position, and
extensor muscles of the wrist and fingers are while maintained in this position, the proximal end
paralyzed (see Figs. 3III, 8). of the flexor superficialis tendons is sutured to the
These patients do not have any kind of hand distal end of the flexor digitorum profundus
release and grasp pattern. Sensory function is tendons with latero–lateral tenorrhaphies. The
usually seriously affected. Myostatic contracture pronator teres is released and the flexor tendons
is frequent with time because of the severity of the of the wrist are ‘‘Z’’ lengthened. This procedure
spasticity and permanent flexed position of the was indicated initially by the author to correct
wrist and digits. pronounced flexion contractures of the wrist and
The best treatment for these patients is to fingers in severe segmentary arthrogryposis, but
release all the upper limb contractures at the the technique also can be indicated in severe group
elbow, forearm, wrist, fingers, and thumb. This is III cases of spastic hand in cerebral palsy. Braun et
accomplished by multiple tenotomies—with mus- al described in 1974 [18] a similar procedure in
cular continuity—of all the contracted muscles. cases after cerebrovascular lesions of the brain.
If the tenotomies are insufficient because of the
great severity of spasticity or myostatic contracture
2-Release of flexion contracture of the elbow
of the finger flexor muscles, the author indicates
a special technique described in 1957 [17] to release Flexion contracture of the elbow is a common
severe contracture of the long flexor tendons of the finding in the spastic hemiplegic upper limb and
fingers. The procedure consists of sectioning the frequently is influenced by emotional stimuli.
flexor superficialis tendons distally, near the wrist, Correction of marked flexion contracture of
and the flexor digitorum profundus tendons the elbow is an excellent indication, because it
proximally at the mid-forearm level. The fingers improves the appearance and function of the
622 E.A. Zancolli / Hand Clin 19 (2003) 609–629

upper limb. Aponeurotic release of the medial brachioradialis [21] (see Fig. 12B), by tendon
epicondyle muscles contributes to correction of plication [20], or by tenodesis of the abductor
the elbow contracture. Additional correction is pollicis longus. In the latter, the proximal end of
obtained by tenotomy in continuity or ‘‘Z’’ the abductor pollicis longus is fixed around the
lengthening of the biceps tendon (see Fig. 12A). distal tendon of the brachioradialis [6]. The best
Occasionally the lacertus fibrosus may be results are obtained by tendon transfers.
sectioned.
Adduction–flexion contracture (thumb-in-palm de-
3-Release of pronation contracture of the forearm formity). This contracture depends on the spas-
ticity of the adductor pollicis and flexor pollicis
Mild cases of forearm pronation spasticity that longus muscles [22]. The most frequently used
reduce passively preoperatively are improved by reconstructive procedures for this deformity are:
simple division of the distal tendon of the pronator (1) release of the origin of the adductor pollicis
teres (see Fig. 12A). In severe contractures the from the third metacarpal, (2) lengthening of the
distal tendon of the pronator teres is rerouted flexor pollicis longus at its musculotendinous
around the radius through the interosseous junction in the forearm, preserving the muscular
membrane (procedure of Tubby [19]) (see Fig. continuity, (3) translocation of the extensor
12C). pollicis longus toward the radial aspect of the
In three adolescent patients of the author’s wrist, and (4) reinforcement of the abductor
series, affected by severe and fixed pronation pollicis longus and extensor pollicis longus by
contracture, the distal end of the ulna was tendon transfers (eg, motors: brachioradialis,
dislocated dorsally. In these cases it was necessary palmaris longus). It is important to maintain
to perform a complete release of the contracted function of the flexor pollicis longus. Overcorrec-
interosseous membrane, excision of the ulnar tion should be avoided. When reinforcement of
head, and a distal radioulnar fusion proximal to the extensor pollicis longus is indicated but
the growing cartilage of the distal radius. This spasticity of the flexor pollicis longus is mild, this
technique fixes the forearm in 10 of pronation, tendon is not lengthened.
which is a useful functional position for the hand.
Stabilization of the metacarpophalangeal joint of
4-Correction of thumb deformity the thumb. This stabilization is indicated in both
types of thumb deformities when the joint is
Surgery is indicated to hold the thumb out of hypermobile in hyperextension (more than 20 ).
palm during grasp and to permit lateral pinch. The most frequently indicated procedures are
The surgical technique indicated depends on the joint fusion or capsulodesis through sesamoid-
type of deformity adduction, or adduction–flexion metacarpal synostosis.
contracture (see Fig. 2). In metacarpophalangeal fusion in children,
Adduction contracture depends on the spas- damage to the epiphyseal line can be avoided by
ticity of the adductor pollicis muscle. There is an the use of transitory thin fixation pins. Meta-
adduction contracture of the first metacarpal. The carpophalangeal joint fusion alone, without prop-
metacarpophalangeal and interphalangeal joints er release of flexion and adduction spasticity and
are not flexed. Correction is obtained by a prox- without reinforcement of the extensor muscles,
imal release of the adductor pollicis muscle and does not in itself eliminate the thumb-in-palm
reinforcement of the abductor pollicis longus. deformity.
Release of the adductor pollicis is obtained by Metacarpophalangeal capsulodesis was de-
sectioning its origin from the third metacarpal scribed by the author in 1968 (see Fig. 102B
through a palmar incision, parallel to the proxi- caption) [4]; 1979 [6], and by Filler et al [23]. In
mal palmar crease (Mateu’s technique) [20] (see this technique, the radial sesamoid is fixed to the
Fig. 12A). neck of the first metacarpal in 10 of flexion
In this type of thumb deformity (the same as in (sesamoid-metacarpal synostosis) (Fig. 14). The
the thumb-in-palm deformity), release of the origin joint is immobilized for 6 weeks.
of the lateral thenar muscles is not indicated,
because there is not hypertonicity of the lateral
Swan-neck deformity
thenar muscles in the classic spastic deformity.
Reinforcement of the abductor pollicis longus Swan-neck deformity can be present in the
usually is accomplished by transfer of the hands of patients with spastic cerebral palsy
E.A. Zancolli / Hand Clin 19 (2003) 609–629 623

Fig. 14. Sesamoid-metacarpal synostosis. (A) Radial side incision in the thumb over the MP joint. (B) The radial
sesamoid without its cartilage (1) is fixed to the neck of the first metacarpal with a thick suture. Cortical bone of the
metacarpal is partially excised (2). The volar plate is preserved. (C) The metacarpophalangeal joint is maintained in a few
degrees of flexion through the tension given to the volar plate (capsulodesis).

[24,25]. This deformity depends on the effect of of the dysfunction of the extensor apparatus. The
a permanent and pronounced flexed position of proximal interphalangeal joint frequently locks in
the wrist and the pull of the long extensor tendons extension and the ability and force of pinch and
during the patient’s efforts to open the hand (see grasp are impaired.
Fig. 9). These conditions are present particularly When the fingers show a permanent and severe
in group II of spastic flexion–pronation deform- swan-neck deformity after the release of the
ity. Under these functional conditions, a progres- flexion spasticity of the wrist and fingers after
sive stretching of the finger’s PIP joint volar plate the first surgical procedure, a surgical correction
is produced, associated with a flexion deformity of of the finger deformity is indicated in a second
the distal interphalangeal joint as the consequence surgical stage. This is an uncommon indication in
624 E.A. Zancolli / Hand Clin 19 (2003) 609–629

spastic cerebral palsy patients [3]. It is a frequent apparatus into the flexor tendon sheath. This
observation that swan-neck deformities improve technique is shown in Fig. 15. The first step of the
spontaneously with correction of flexion contrac- operation consists of freeing the extrinsic lateral
ture of the wrist because this decreases the extensor band of the affected fingers (radial side)
traction of the long digital extensor tendons over between the middle third of the proximal phalanx
the middle phalanx. If surgery is indicated, the and the middle third of the middle phalanx.
author prefers his procedure described (since The distal end of this tendinous sling remains
1975) as the ‘‘sling’’ operation [5,26–28]. attached to the extensor apparatus, and the
The procedure consists of the translocation of proximal end is continuous with the central
the radial side lateral band of the extensor extrinsic extensor tendon and with the intrinsic

Fig. 15. Correction of severe swan-neck deformity (technique). (A) Mid-lateral longitudinal incision on the radial side of
the finger. (B) The lateral band (1) is released between the middle of the proximal phalanx and the middle of the middle
phalanx. The proximal end of the released band remains in continuity with the lateral slip of the extensor tendon and the
lateral band of the intrinsic tendons. The flexor tendon sheath is opened at the proximal PIP joint level (2). (C) The
released lateral band (3) is translocated to the volar part of the finger and placed between the volar plate (5) and the
flexor superficialis tendon (6). Two strong stitches (4) join the volar plate and the chiasma of Camper, distal to the PIP
joint level. These sutures maintain the lateral band volarly to the joint. (D) After the procedure, the finger must extend
passively up to almost neutral extension. The suture of the volar plate to the flexor superficialis represents a pulley for the
transferred lateral band (7). During active finger extension, the transferred band produces a simultaneous stabilization of
the PIP joint and an extension of the distal interphalangeal joint. The lateral band in its new position is shortened. The
normal use of the finger produces with time the relocation of the opposite lateral band to its normal position.
E.A. Zancolli / Hand Clin 19 (2003) 609–629 625

lateral band (radial interosseous and lumbrical). 3. Speed, skill, voluntary control, and coordi-
The second step consists of opening the flexor nation for prehension are functions examined
tendon sheath and exposing the flexor tendons at by the pick-up test and by manipulating
the PIP joint level. In a third step, the lateral band objects. Manipulation of toys by children can
is rerouted palmar to the joint and introduced help the surgeon make decisions as to
between the palmar plate and the flexor superficialis indication for reconstructive procedures in
tendon (Camper’s chiasma). The lateral band is an attempt to improve function and cosm-
maintained in this position by two strong separate esis. Function between both hands is studied.
sutures (with the finger in 5 of PIP flexion) that 4. Grasp and pinch strength. The grip strength
unite the lateral borders of the tendinous chiasma is measured with a dynamometer. A sphyg-
and the palmar plate. The transposed lateral band momanometer may be used to record grip
should not be included in the suture. These sutures strength in weak hands. A pinch meter is used
are located distally to the PIP joint level at the base to evaluate pinch strength.
of the middle phalanx. The tendon sheath is closed. 5. Activities of daily living. These activities in-
If the tension of the tendinous sling is correct, the volve hygiene, dressing, writing, and feeding.
finger extends passively to 5 of PIP flexion. If PIP 6. Active and passive range of motion of all the
flexion is greater than 5 , the freeing of the lateral upper limb joints (shoulder, elbow, forearm,
band is extended at its proximal or distal ends to wrist, fingers, and thumb) are examined. The
reach the desired PIP position. If there is some range of motion should be recorded on the
tendency to PIP joint recurvatum, stitches are principle that the neutral position equals 0 .
applied at either end to tighten the sling. Flexion The patient is asked to straighten the fingers
greater than 5 of the PIP joint may produce and to make a fist to obtain a global idea of
a boutonniere deformity. motion of all digits and to note limited
This technique represents the reconstruction motions or abnormal postures.
of an active retinacular ligament. The activation
of the tendinous sling by the action of the
extensor tendons and the intrinsic muscles Results
produces complete finger extension and corrects
Results after surgery can be grouped as good,
the hyperextension of the PIP joint. The finger
fair, and poor in relation to the assessment of
is immobilized in slight flexion for 1 week
hand function.
before active exercises begin. The results of this
procedure have been encouraging. The pro- Good
cedure is contra-indicated in cases with intrinsic Complete digital extension with less than 20
paralysis (claw-hand). wrist flexion. In the best of cases the wrist
dorsiflexes with complete digital extension.
Complete elbow extension and almost com-
Assessment of hand motor function plete active forearm supination.
Good lateral pinch and grasping (thumb-in-
Grouping of patients for results of surgery is
palm deformity corrected).
obtained by the evaluation of different upper limb
Good voluntary control of muscles.
functions [10]. These evaluations include:
The affected limb is predominantly used as an
1. Ability to open the hand. This is evaluated by assistant of the normal side.
using wood discs of different sizes (6, 8, 10, Fair
12, 14, 16, and 18 cm); determining hand Complete digital extension with more than 20
placement over the table; and measuring the of wrist flexion (reduced with respect to the
degrees of wrist flexion that enable the patient preoperative value).
to extend the fingers. Partial elbow extension and forearm supi-
2. Grasp and pinch patterns. The ability to nation.
grasp is evaluated through the use of spher- Weak and partial lateral pinch and grasping.
ical balls (5, 8, 10, and 12 cm) and cylinders The limb is used only as an assistant.
(3, 5, 8, 10, and 12 cm). Tip, pulp, lateral Poor
pinches, and chuck three-digit pinch are Hand function and deformities not improved
evaluated during manipulation of different with respect to the preoperative condition
objects. (passive hand).
626 E.A. Zancolli / Hand Clin 19 (2003) 609–629

Fig. 16. (A) A group IIB of infantile spastic hemiplegic hand in a 17-year-old boy. Voluntary finger flexion was possible.
The right hand was dominant. (B) Fingers extend actively with a flexion spasticity of the wrist of 70 . Surgical program
shown in Fig. 12 was indicated. (C) Postoperative; shows that the fingers extend now with only 25 of wrist flexion. The
thumb extends actively. (D) Complete active closure of the hand. Thumb-in-palm deformity has been corrected. (E)
Excellent lateral pinch was obtained. (F) Hand posture and function obtained during eating.
E.A. Zancolli / Hand Clin 19 (2003) 609–629 627

The author considers that good and fair results fair and 7 poor. In most cases of group I and II, the
represent a satisfactory improvement for these patient or the family believed there was substantial
patients. postoperative improvement in function and cosm-
In the author’s surgical series of 91 spastic esis of the hand (Figs. 16, 17).
patients, 47 cases were evaluated for their final Three pure athetoid patients who had soft
result. Twenty-nine were females. The age at tissue procedures had a poor result. The initial
operation was after 7 years in most cases. deformities were replaced with other deformities.
According to the classification presented before,
Summary
the number of patients was: group I, 8 cases,
group II, 31 cases, and group III, 8 cases. Selected spastic patients with cerebral palsy
The results were: group I, 7 good and 1 poor; can be helped by peripheral reconstructive surgery
group II, 19 good, 10 fair, and 2 poor; group III, 1 of the upper limb.

Fig. 17. Spastic infantile hemiplegia (group IIA) combined with athetosis in a 15-year-old girl. (A) Hand deformity when
attempting hand release. (B) Hand closure preoperatively. Active wrist extension was possible. (C,D,E) Postoperative
result after muscle rebalance. Good digit extension, hand closure, and lateral pinch was obtained. The thumb-in-palm
deformity was corrected. The FCU was not transferred to the wrist extensor because it was a group IIA.
628 E.A. Zancolli / Hand Clin 19 (2003) 609–629

cerebral palsy. New York: Harper & Row; 1975.


p. 221–57.
[3] Swanson AB. Surgery of the hand in cerebral palsy
and muscle origin release procedures. Surg Clin N
Am 1968;48:1129–38.
[4] Zancolli EA. Structural and dynamic bases of hand
surgery. Philadelphia: JB Lippincott; 1968.
[5] Zancolli EA. La operación del ‘‘asa’’ en la
corrección de la deformidad en ‘‘cuello de cisne.’’
Primer Congresso Nacional de la Sociedad Espa-
ñola de cirugia de la mano. Octobre 17–19, 1975.
Bilbao, España (unpublished).
[6] Zancolli EA. Structural and dynamic bases of hand
surgery. 2nd edition. Philadelphia: JB Lippincott;
1979.
[7] Blumel I, Eggers GWN, Evans EB. Genetic
metabolic and clinical study on 100 cerebral palsied
patients. JAMA 1960;174:860–72.
[8] Samilsom SL, Morris JM. Surgical improvement of
the cerebral-palsied upper limb. J Bone Joint Surg
Fig. 17 (continued ) 1964;46A:1203–16.
[9] Green WT. Tendon transplantation of the flexor
carpi ulnaris for pronation-flexion deformity of the
wrist. Surg Gynecol Obstet 1942;75:337–42.
Although surgery cannot make a limb that was [10] Zancolli EA, Zancolli ER. Surgical rehabilitation of
functionally poor into a perfect one, it can greatly the spastic upper limb in cerebral palsy. In: Lamb
improve the preoperative condition. Most poor DW, editor. The paralysed hand. New York:
surgical results are caused by incorrect selection of Churchill Livingstone; 1987. p. 153–68.
patients or poor execution of surgical procedures. [11] Twitchell TE. Sensation and the motor deficit in
The worst mistake is to perform soft tissue cerebral palsy. Clin Orthop 1966;46:55–62.
procedures—tendinous release or tendinous trans- [12] Zancolli EA, Zancolli ER. Surgical management of
fers—on a patient with pure athetosis. The results the hemiplegic spastic hand in cerebral palsy. Surg
in these cases are unpredictable and often fail. The Clin N Am 1981;61:395–406.
[13] Page CM. An operation for the relief of flexion
same concept is applied to rigidity, hypotonia,
contracture in the forearm. J Bone Joint Surg
dystonia, and ataxia. 1923;5A:233–4.
The surgical program is organized according to [14] Inglis AE, Cooper W. Release of the flexor pronator
the type and severity of the deformity (clinical origin for flexion deformities of the hand and wrist
groups). in spastic paralysis. J Bone Joint Surg 1966;48A:
The goals are to correct the deformities and to 847–57.
improve the muscular balance of the hand in one [15] White WF. Flexor muscle slide in the spastic hand.
surgical stage. It must be remembered that spastic The Max Page operation. J Bone Joint Surg
muscle cannot be used for tendon transfer with 1972;54B:453–9.
the same efficiency as in patients with a flaccid [16] Green WT, Banks HH. Flexor carpi ulnaris trans-
plant: its use in cerebral palsy. J Bone Joint Surg
paralysis. Results were satisfactory in 92% of
1962;44A:1343–52.
cases of groups I and II. If group III is included, [17] Zancolli EA. Un nuevo método de corrección de las
the percentage of satisfactory results reduces sig- contracturas congénitas de los músculos flexores
nificantly. digitales (alargamiento intetendinoso). Prensa Méd
Argentina 1957;44:279–81.
[18] Braun RM, Guy TV, Roper B. Preliminary
References experience with superficialis-to-profundus tendon
transfer in the hemiplegic upper extremity. J Bone
[1] Goldner JL. Reconstructive surgery of the hand in Joint Surg 1974;52A:466–72.
cerebral palsy and spastic paralysis resulting from [19] Vulpius O, Stoffel A. Orthopädische Operations
injury to the spinal cord. J Bone Joint Surg Lehre. Stuttgart: Ferdinang Enke; 1920.
1955;37A:1141–54. [20] Matev I. Surgical treatment of the spastic ‘‘thumb-
[2] Goldner JL. The upper extremity in cerebral palsy. in-palm’’ deformity. J Bone Joint Surg 1963;
In: Samilson RL, editor. Orthopaedic aspects of 45B:703–8.
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[21] McCue FC, Honner R, Chapman WC. Transfer [25] Swanson AB. Surgery of the hand in cerebral palsy.
of the brachioradialis for hands deformed by In: Flynn JE, editor. Hand surgery. 3rd edition.
cerebral palsy. J Bone Joint Surg 1970;52A: Baltimore: Williams & Wilkins; 1982. p. 476–88.
1171–80. [26] Zancolli EA. Management of boutonniere and
[22] Keats S. Surgical treatment of the hand in cerebral swan-neck deformities. In: Program of the 25th
palsy: correction of thumb-in-palm and other congress of the Japanese Society for Surgery of the
deformities. Report of nineteen cases. J Bone Joint Hand. Tokyo: 1982.
Surg 1964;47A:274–84. [27] Zancolli EA. Panel on soft tissue reconstruction in
[23] Filler BC, Stark HH, Boyes J. Capsulodesis of meta- rheumatoid arthritis. In: Program of the 41st
carpophalangeal joint of the thumb in children with Annual Meeting of the American Society for
cerebral palsy. J Bone Joint Surg 1976;58A:667–70. Surgery of the Hand. New Orleans: 1986.
[24] Swanson AB. Surgery of the hand in cerebral palsy [28] Zancolli EA. Surgical correction of flexible swan-
and the swan-neck deformity. J Bone Joint Surg neck deformity. Volar translocation of the radial
1960;42A:951–64. lateral band. Curr Orthop 1991;5:230–2.
Hand Clin 19 (2003) 631–648

The upper limb in cerebral palsy: surgical management


of shoulder and elbow deformities
A. Landi, MDa,*, S. Cavazza, MDb, G. Caserta, MDa, A. Leti Acciaro,
MDa, S. Sartini, MDc, M.C. Gagliano, MDa, M. Manca, MDb
a
Unit of Hand Surgery and Microsurgery, Policlinico of Modena, Via del Pozzo, 71 41100, Modena, Italy
b
Unit of Rehabilitation, St. Giorgio Hospital, Ferrara, Italy
c
Unit of Rehabilitation, Policlinico of Modena, Via del Pozzo, 71 41100, Modena, Italy

The population of patients affected by ac- fined to the segments below the elbow. Scant
quired spasticity is large. Apart from cerebral attention has been given in the past to the
palsy (CP), numerous different conditions ulti- classification and treatment of shoulder deformity
mately can lead to spasticity of the upper limbs: [6] and spastic deformities of the elbow [7,8].
cerebrovascular accidents (CVA), traumatic brain
injuries (TBI), brain tumors, and spinal cord
The shoulder
injuries (SCI) (Table 1).
Injury to the upper motor neuron following Hypotonic and hypertonic phases follow cere-
supraspinal trauma may be defined as a disorder bral lesions. Initially there is complete muscle
of motor control attributable in part to the loss of flaccidity with a total lack of voluntary control.
cortical control of the spinal cord, characterized In this phase the risk for subluxation of the
by weakness, impaired coordination (negative scapulohumeral joint is high, and indeed is
signs), spasticity, increased tendon jerks, and observed in 66%–92% of hemiplegic patients [6].
release of primitive cutaneous-muscular reflexes, There are three forms of subluxation of the
such as the Babinski response (positive signs) [1]. hemiplegic shoulder: anterior, superior, and in-
The primary lesion often is associated with ferior. Inferior subluxation is the most frequent,
secondary lesions of the muscle and joint that act whereas anterior and superior subluxations are
in concert to alter the movements or posture of observed in a smaller percentage of patients and
the subject. Secondary lesions include muscle often in a later phase of recovery [6].
retraction, bone deformities, and capsular re- A few days or weeks following supraspinal
traction from disuse; these are elements that must damage the patient progressively regains partial
be considered when planning the therapeutic control of the muscles of the scapular girdle.
program. It is now known that increased passive Voluntary recruitment of the muscle is usually
muscle tension is generated by changes in slow, as is the capacity to relax the muscle at the
rheologic properties (viscoelastic and plastic end of contraction. The subject has great difficulty
properties) [2]. in activating simple movements selectively and
Surgical attention for the upper limb in is able to reproduce only simple motor patterns.
cerebral palsy traditionally has been concentrated The force generated is generally low and is not
on the distal upper limb segment. The universally sufficient to achieve antigravitational movement.
adopted classification of Zancolli [3] and its A light muscular activity or emotional stress can
subsequent modifications [4,5] usually are con- trigger an ‘‘associated reaction’’ with activation of
muscle patterns at the shoulder girdle.
* Corresponding author. Some motor units are under voluntary control
E-mail address: landi_antonio@virgilio.it (A. Landi). and other units are flaccid or hypertonic to passive
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00062-3
632

Table 1
Lindon Lindon Pinzur Pinzur
Target pre- post- pre- post-
Patient Age Sex Etiology Diagnosis Segment Site Procedures (group) operative operative operative operative
1. P. S 27 M T.B.I. Tetra S+ E L MDr,Bt,BCr,BRpr Hygienic 6 6 1 1
2. M. M 24 M T.B.I. Tetra E L Bt,BCt,BRpr,Er Hygienic 6 6 1 1
3. V 52 F C.V.A. Hemi S+ E R PMr,Bbr,CBr Hygienic 6 6 0 0
4. DE 27 M C.P. Tetra S+ E+F L PMr,Bbr,BRdr,FCUr,FCRr Hygienic 6 6 0 1
5. PT 24 M T.B.I. Hemi S+ E+F L PMr,LDr,TMr,Br,BRpr,FCRt, Hygienic 6 6 1 2
FCUt,STP
6. OM 30 F C.P. Hemi S + E R PMr,CBr,Bt,BRpr Hygienic 6 6 0 2
7. CL 73 F C.V.A. Hemi S + E L PMt,Bbr,BCr,LDt,TMt,BRpr,MEMbr Hygienic 6 6 0 1
8. BK 28 F C.V.A. Hemi S + E R PMr,SHBr,CBr,LDr,TMr,MEMbr,BRdr Hygienic 5 5 1 2
9. CE 25 M T.B.I. Hemi S + E L PMr,Bbr,MEMbr,BRpr Hygienic 6 6 0 0
10. CR 41 F T.B.I. Tetra S + E L PMr,LDr,TMr,SHBr,CBr,Bt,BCt, Hygienic 6 6 0 0
BRpr,FCUt
11. B. L 62 M TB.I. Hemi E+F R Bt,BCt,MEMbr Hygienic 6 6 0 0
12. C 33 F C.V.A. Hemi S L PMt,SHBr,CBr,ADr Hygienic 6 6 0 1
13. ME 37 M T.B.I. Hemi E L Bzlnt,BCr,MEMr Hygienic 6 6 0 0
14. MV 46 F C.V.A. Hemi S L PMt,LDt,TMt,SHBr,CBr Hygienic 6 6 2 3
15. BA 21 M T.B.I. Hemi E+F L Bzlnt,BRpr,MEMr,ECRLt, Hygienic 6 6 2 2
ECRBr,FCRt
16. FS 28 M T.B.I. Hemi E L Bzlnt,LEMr,PLt Hygienic 6 6 1 1
17. SF 23 M T.B.I. Tetra E L Bt,BRpr Hygienic 6 6 0 0
A. Landi et al / Hand Clin 19 (2003) 631–648

18. FR 50 M C.V.A. Hemi E+F L Bzlnt,BCt,BRpr Hygienic 6 6 0 1


19. MD 30 M T.B.I. Tetra S+E+F R PMr,LDr,TMr,Br,BRpr,FCRt, Hygienic 6 6 1 1
FCUt,STP
20. BN 37 M T.B.I. Tetra S+E L PMt,Bt,BCt,SHBr,CBr,TMt,MEMr Hygienic 6 6 0 0
21. CS 32 M T.B.I. Hemi S+E+ F L PMt,ADr,MEMbr Hygienic 6 6 0 0
22. BM 17 M T.B.I. Tetra S+E+ F L PMt,SHBr,CBr,Bt,BRpr,FRCr,FCUr Hygienic 6 6 1 1
23. FE 37 F T.B.I. Hemi S+E L PMt,Bt,MEMr Hygienic 6 6 1 1
24. G. S 27 M T.B.I. Tetra E+F L BRpr,FCUt, FCRt,PRC Functional 5 5 2 3
25. T.R 27 F T.B.I. Hemi E R Er,UNant Functional 5 3 1 1
26. L 26 M T.B.I. Hemi E+F R MEMr,UNant, Er, HOr Functional 5 4 2 3
27. PF 22 M T.B.I. Hemi E R Bfrltn,BCt,Er, HOr Functional 4 3 1 3
28. De D 26 M T.B.I. Hemi S+E+ F R PMr,SHBr,CBr,LDr,TMr, MEMbr Functional 6 5 0 2
29. VG 52 F C.V.A. Hemi S+E+ F L PMr,SHBr,CBr,MEMbr Functional 4 3 2 3
30. MM 34 M T.B.I. Tetra E L Br,BRpr,Er Functional 4 4 2 2
31. PG 33 M T.B.I. Hemi S+F L PMr,FCUr,FCRr,PINd Functional 4 4 2 3
32. FL 56 M C.V.A. Hemi S R PMr,CBr,SHBr Functional 5 2 2 5
33. OG 25 F C.P. Tetra S+E+F L PMr,ADr,Bzlnt,FCRt Functional 5 4 2 3
34. RC 29 M T.B.I. Tetra E+F R MEMbr, AbPLtoECRB Functional 6 4 1 2
35. C.M 24 M C.V.A. Hemi E+F L MS,IMr,Grilli Functional 5 5 2 3
36. MA 22 M T.B.I. Hemi E L Bzlnt,BCt,Er,UNant,HOr Functional 5 4 3 4
37. TD 20 M Enc. Hemi E+F L Bzlnt,BCr,BRpr,MEMr,LEMr, Functional 6 5 3 3
FCRtoECRB, Caps
38. BA 18 M C.P. Hemi S+E+F L PMr,SHBr,CBr,BRdr,MEMr, PRC Functional 5 4 3 4
39. VF 37 M T.B.I. Hemi S+E R PMr,Er Functional 5 4 2 3
40. M. A 47 M C.V.A. Hemi E+F L Bt,MEMbr Mixed 5 5 2 2
41. C. D. 23 M T.B.I. Tetra S+E R PMt,Bdr,MEMr Mixed 6 6 0 1
42. MM 51 M C.V.A. Hemi S+E+F L PMt,Bbr,ADr,MEMr,BRdr Mixed 5 5 1 1
43. NV 52 F C.V.A. Hemi S+E L PMt,Bbr,ADr,BRpr Mixed 5 5 1 2
44. BM 30 F T.B.I. Hemi E+F L Bdr,BRdr,FCUt Mixed 6 5 2 2
45. Z. A 21 M T.B.I. Tetra E+F L Bbr,BRdr,FCUt,FCRt Mixed 6 4 2 2
46. VM 26 M T.B.I. Hemi E L Ancr,BRpr, MEMr Mixed 5 5 1 1
47. S .P 25 M C.V.A. Hemi E R Bt Mixed 6 6 1 1
48. FL 25 M T.B.I. Tetra E R Tlnt,Er Mixed 6 5 2 3
49. IM 43 F C.V.A. Hemi S+E L PMt,SHBr,CBr,MEMr Mixed 6 4 1 3
50. CB 18 M C.V.A. Hemi E+F R Bbr,MEMr Mixed 6 6 4 4
CP, cerebral palsy; CVA, cerebral vascular accident; TBI, traumatic brain injury; Enc, encephalitis; S, shoulder; E, elbow; F, forearm; Hemi, hemiplegia; Tetra, tetraplegia;
L, left; R, right.
See Table 3 for explanation of abbreviations of procedures.
A. Landi et al / Hand Clin 19 (2003) 631–648
633
634 A. Landi et al / Hand Clin 19 (2003) 631–648

stretching, with the capacity for voluntary con- lesion observed in vascular lesions of the middle
trol. These lead to different postures of the cerebral artery, there are also an appreciable
shoulder. number of patients with a lesser degree of motor
damage who show a certain capacity for volun-
Scapulohumeral internal rotation and slight tary movements that go beyond the patterns
abduction described. These patients have the capacity to
abduct and adduct or carry out flexion–extension
This posture generally is associated with of the shoulder while maintaining the elbow
a subcontinual activation of the upper bundles extended, to maintain the shoulder in abduc-
of the trapezius and supraspinatus muscles and of tion–anteropulsion against gravity and perform
the three heads of the deltoid, which the patient flexion–extension of the elbow, and to produce
often is not able to activate selectively. The subject voluntary external rotation of the shoulder.
generally is able to produce movements of in- Consideration also should be directed to the
complete abduction, with partial anteroposition shoulder joint, as rotator cuff tears may occur
of the shoulder and a synergistic pattern with the occasionally and should be recognized as a possi-
elbow in flexion. This pattern, in patients with ble cause of non-neuropathic pain at this level.
severe impairment, can be associated with anterior Heterotopic ossifications (HO) may be associated
or superior subluxation of the scapulohumeral with head injuries and usually form inferomedially
joint and of postures that are maintained over to the joint but might locate posteriorly and
a wide range of voluntary movements and gait, usually follow the teres muscles. When surgical
assuming patterns of spastic dystonia. A limited removal is considered, care must be exercised to
number of these patients, especially those with identify the axillary nerve. Furthermore, the
deep cerebral lesions associated with cranioence- trauma concurrent with brain injury may have
phalic trauma, assume a posture with the shoulder caused complex fractures that might have been
held in abduction and slight extension. missed in the initial resuscitation phase. Malunion
of the proximal humerus and of the greater
tuberosity are possible causes of underlying non-
Scapulohumeral internal rotation and adduction
neuropathic pain that also can lead to degenera-
This posture is associated frequently with tive arthritis [9].
a subcontinuous activation of the pectoralis
major, latissimus dorsi, and teres major muscles.
Activation frequently has features of associated
The elbow
reaction of the superior bundles of the trapezius,
supraspinatus and anterior deltoid muscle. In Many patients present with hypertonus of the
these cases, only modest anteroposition of the flexor muscles of the elbow. The extensor muscles
shoulder is generally possible in combination with are inactive or weak, with disorders of phasic
elbow flexion and wrist and finger flexion. Closely contraction.
associated with shoulder adduction is limited The posture most commonly assumed is
forward flexion, making it difficult to position flexion of the elbow associated with pronation
the hand appropriately for functional activities. or supination of the forearm. Usually flexor
Dynamic EMG studies are extremely useful in muscle control is poor and may be associated
identifying dyssynergic extensor muscle activity with spastic dystonia or muscular retraction.
and in documenting appropriate firing of the At dynamic EMG evaluation, spastic contrac-
flexor muscles. The muscles contributing to tion of the brachioradialis muscle often is seen,
spastic extension deformities include the posterior whereas the biceps and brachialis muscles contract
deltoid, long head of the triceps, teres major, and as an associated reaction and are under volun-
latissimus dorsi [9]. tary control with prolonged and continuous
The lowered posture of the shoulder is caused activation.
by the need to maintain the plegic hemisoma in The study of the capacity to control the flexor
relative contraction (the pelvic hiking effect) to and extensor muscles of the elbow is useful for
permit improved advancement of the plegic lower evaluating the degree of voluntary motion and
limb. compulsory postures in flexion of the elbow.
In addition to this more severe condition Occasionally posterior dislocation of the radial
associated with a focal cortical–subcortical brain head might develop in cerebral palsy, though
A. Landi et al / Hand Clin 19 (2003) 631–648 635

rarely requiring treatment [8]. The incidence of and a manual dexterity scale for single hand
HO at the elbow is approximately 90% in (Jebsen test) and bimanual (De Renzi test for
combined head and elbow injury (Fig. 1). HO ideomotor apraxia) tasks [13]. In particular,
often involves the collateral ligament and might global functioning was assessed using a qual-
contribute to ulnar palsy (Fig. 1). Surgical re- itative scale such as the Lindon score [4,5].
moval usually has a positive effect on outcome [8] The capacity to control the shoulder and
(Fig. 1). elbow voluntarily was evaluated using the
Elbow joint stiffness occasionally can be hand placement scale proposed by Pinzur,
observed in the absence of HO, and joint release modified by the authors’ group [4,5], whereas
for functional and hygienic purpose should be the capacity for efficient handgrip was mea-
considered. sured with the Sollerman test as modified by
Eliasson [4,5].
 Evaluation of cognitive functioning (Mini
Indications for surgery and assessment methods Mental State Examination), attention, and
memory, in addition to clinical assessment of
It is necessary to assess possible surgical
severe neglect or apraxia.
candidates on multiple occasions. It is unusual
 The tactile sensory, proprioceptive, and dis-
to operate on patients with cerebral palsy younger
criminative level were evaluated by means of
than age 5 years and is more common in
the Fugl-Meyer [14] score for tactile discrim-
adolescents, but is not contraindicated in the
ination and proprioception and of the
adult. Following CVA it is necessary to wait
Moberg test for two-point discrimination.
a minimum of 1 year for clinical stabilization and
 Videotaping of standardized movements in-
more than 2 years following spastic palsy as
volving reaching and grasping is beneficial.
a result of head injury.
 The passive range of motion of the elbow in
Severe behavioral disorders, severe and wide-
flexion and extension and of the shoulder
spread dystonia, and presence of choreoathetotic
in abduction and external rotation is
movements, myoclonus, or disorders of coordina-
documented.
tion are contraindications to surgery. Focal
 The modified Ashworth scale [15] is used for
dystonia is in itself not an absolute contrain-
assessment of the flexors of the elbow and for
dication, especially if it responds to local phar-
abduction and external rotation of the
macologic treatment with botulinum toxin or
shoulder.
diagnostic nerve blockade. Severe sensory deficits
do not prevent surgery but gains in functional
outcome are limited. The surgeon must specifically evaluate the
Preoperative assessments and outcome meas- following:
ures include the following:  Differential diagnosis between myostatic re-
 Identification of the priority placed by the traction and spastic hypertonus by means of
patient or caregiver on posture by means of clinical tests, diagnostic nerve blocks, and
a questionnaire derived from the Canadian dynamic EMG during passive mobilization.
Occupational Performance Measure (COPM)  Capacity for voluntary control of muscle
[10] in which the patient or caregiver defines antagonist to those in myostatic retraction
the most relevant problems of the upper limb by means of dynamic EMG performed during
and the degree of satisfaction with motor simple voluntary movements.
performance.  Clinical study of the movements associated
 Assessment of the level of global disability by with pathologic synergisms with the contri-
means of the FIM scale [11] and Disability bution of dynamic EMG studies recorded
Rating Scale. Considering the low sensitivity during complex movements and minimum
of the global rating scale for assessing force or during walking.
treatment for segmental spastic hypertonus,
the authors employed the scale proposed by These evaluations allow assessment of, for
Bhakta [12] to evaluate the impact of spastic each muscle studied, the prevalence of retraction,
hypertonus on disability. spastic hypertonus, and the level of coactivation
 Evaluation of the functional level of the or dyssynergy among antagonistic muscles, in
upper limb by means of a qualitative scale addition to the degree of voluntary control of the
636 A. Landi et al / Hand Clin 19 (2003) 631–648

Fig. 1. (A,B) Right hemiplegia following a road traffic accident (RTA) with subsequent coma for 1 month. Elbow
ankylosed at 85 . (C) Radiograph showing the posterior location of HO at the elbow. (D) Corresponding CT scan. (E)
Intraoperative findings following HO removal, MEMr, and ulnar nerve transposition. Arrow points to ulnar nerve. (F)
Postoperative radiographs. (G,H,I,J) Functional outcome. The patient has gained one score on the Lindon scale.
A. Landi et al / Hand Clin 19 (2003) 631–648 637

Fig. 1 (continued )

muscles in their capacity to contract and relax the muscles were evaluated at the shoulder: pectoralis
motor units. Moreover, the degree that the muscle major, three heads of the deltoid, supra and
is influenced by movements associated with infraspinatus, latissimus dorsi, teres major, and
minimum forces is evaluated. long head of the triceps.
The following muscles were studied at the
elbow: biceps, triceps, brachialis, long, middle,
Dynamic EMG study
and lateral heads of the triceps, and anconeus.
The dynamic EMG study is performed using The last one has been studied particularly because
fine wire electrodes in the muscles examined and of the possible interference with active prono-
electronic goniometers at the elbow joint. The supination. Each muscle was studied to evaluate
patient is examined in the sitting position, the degree of spastic hypertonus, the capacity for
postured with the flexor muscles at maximum voluntary control, and the appearance of associ-
stretch for 5–10 sec during simple flexor–extensor ated reactions. To study spastic hypertonus,
movements of the elbow joint with a fixed passive stretching movements at different speeds
shoulder, of abduction of the shoulder and were performed, and the muscle activity during
internal and external rotation during a brief task prolonged postures (5 sec) was evaluated with the
of maximal flexion of the contralateral elbow, of studied muscles in the stretched position.
flexion of the homolateral hip, and, when Finally the surgical objectives are established
possible, of a task with walking in place. following the assessments mentioned previously in
To define muscular activation the authors’ which the priorities proposed by the patient and
used the criteria of the Keenan [16] and Mayer caregiver are combined with a realistic assessment
[17], introducing an additional classification cri- of the level of function and use of the limb in
teria for associated movements when studying activities of daily living. It is then possible to place
voluntary control. In particular, the following the patient into one of three functional groups.
638 A. Landi et al / Hand Clin 19 (2003) 631–648

1. Personal hygiene. The patient has no capacity Table 3 documents the surgical procedures that
for functional use of the upper limb in either were performed and the abbreviations that de-
mono- or bimanual functions. Obligatory scribe these. Table 4 describes the shoulder and
function leads to major disability because of elbow procedures that were performed according
pain and difficulty in managing dressing and to the three groups of patients: (1) hygienic, (2)
personal hygiene of the armpit, elbow, and functional, and (3) mixed.
hand. All patients were followed at 6 months by
2. Functional outcomes. The patient has a dis- a medical team consisting of an orthopaedic
crete capacity to use the plegic limb in mono- surgeon, a physiatrist, and a neurologist. The
and bimanual activities. In particular, the patients were evaluated by and compared with the
patient shows at least a certain capacity to use preoperative rating scales, recording the achieve-
the plegic upper limb for digitopalmar grasp- ment of targeted outcomes and observation of
ing and is able to release the grip actively even videos made before and after surgery.
though lacking the capacity to use the limb
directly in single hand activities. Group 1: surgery for personal hygiene
3. Mixed problems. The patient presents a cer-
Twenty-three patients were operated on to
tain degree of passive bimanual function,
achieve improved personal hygiene (8 tetraplegic
though using for daily activities an alternative
and 15 hemiplegic). Twenty-one of the 23 patients
grasp between the arm and chest wall or
were operated on at the elbow for problems
forearm and abdomen or using the closed fist
correlated with contracted posture in flexion. Of
to hold small objects. Problems with the
these 21 patients, 4 had surgery at only the elbow,
obligate posture and personal hygiene prevail.
9 received an associated treatment at the elbow
and shoulder, and 5 had three interventions at the
Clinical and surgical experience shoulder, elbow, and forearm. Of the remaining
three, one had surgery at the elbow and wrist and
The authors have assessed 138 patients treated
two at only the shoulder (Table 1).
surgically for spastic disorders. Fifty of these
The global disability scale of the FIM and the
underwent surgery of the shoulder or elbow
DRS were not modified significantly, whereas the
(Tables 1 and 2). Fifteen patients were tetraplegic,
scale of impact of spastic hypertonus on disability
13 as a result of head injuries and 2 with infantile
(ad hoc scale) showed some change.
cerebral palsy. Thirty-five patients were hemi-
In particular, patients changed significantly
plegic, 17 following head injury, 12 following
globally in the Bhakta rating scales for caregivers,
CVA, 1 following a cerebral infection, and 5 with
from 2.2 to 1.5, whereas no significant changes
infantile cerebral palsy.
were observed in the scales specific for disability.
There were 36 males and 14 females with an
All patients presented at the first intervention
age range of 17–73 years. The mean FIM motor
a level of 6 on the Lindon scale and a value of 0–1
scale score of disability was 66.6 with a mean
on the Pinzur scale. At follow-up, patients
Disability Rating Scale (DRS) of 9.2. Considering
operated on for personal hygiene did not show
the high correlation between the data obtained
a change on the Lindon scale.
with the DRS and FIM motor scale (r = 0.9,
Of the patients with surgery to the shoulder,
P = 0.000), the authors considered the values of
the main problem was related to shoulder
the DRS in this study. Of the 15 tetraplegic
patients, 4 had surgery limited to the elbow, 4 had
surgery of the elbow and shoulder, and 4 had Table 2
Etiology
surgery of the shoulder, elbow, and forearm. The
remaining three had surgery of the elbow and Group 1 Group 2 Group 3
forearm. None were treated for a shoulder prob- Hygienic Functional Mixed Total
lem alone. Of the hemiplegic patients, three had Perinatal (CP) 2 2 3 7
operations on the shoulder alone, seven on the Cerebral 2 1 2 5
elbow alone, one on the shoulder and forearm, aneurism
nine on the shoulder and elbow, nine on the elbow Thrombosis 4 2 1 7
and forearm, and six on the shoulder, elbow, and TBI 4 6 1 11
forearm. Three patients also had surgery for HO TBI with coma 11 4 4 19
Encephalitis — 1 — 1
at the elbow joint.
A. Landi et al / Hand Clin 19 (2003) 631–648 639

Table 3
Legend of the surgical procedures performed on muscles, tendons, and joints, and corresponding abbreviations
t Tenotomy
r Release Proximal release of a muscle at its origin
frlnt Fractional lengthening Surgical technique of lengthening of a myotendineous
unit by a single or multiple tenotomies at the
myotendineous junction
zlnt Z lengthening Surgical technique of lengthening of a myotendineous
unit at the distal tendon
AD Anterior deltoid muscle —
MD Middle deltoid muscle —
PD Posterior deltoid muscle —
LD Latissimus dorsi —
TM Teres major —
PM Pectoralis major —
CB Choracobrachialis —
BC Brachialis —
Anc Anconeous muscle —
IM Interosseous membrane —
STP Superficialis to profundis —
MS Muscle sliding Release of a group of muscles within a muscle compartment
Bbr Biceps bipolar release Biceps fractional lengthening at the short head and at
the myotendineous junction
BRpr Brachioradialis proximal release —
BRdr Brachioradialis distal release Fractional lengthening of brachioradialis at the
myotendineous junction
MEMr Medial epicondylar muscle release —
MEMbr Medial epicondylar muscle Proximal muscle release + fractional lengthening of pronator
Bipolar release teres, Flexor carpi radialis, and palmaris longus
LEMr Lateral epicondylar muscle release —
PRC Proximal row carpectomy —
Er Elbow arthrolysis —
Caps Anterior elbow capsulotomy —
UNant Ulnar nerve anteposition —
PINd Posterior interosseous nerve —
decompression
Grilli — Biceps rerouting
HOr Heterotopic ossification removal —

Table 4 adduction and internal rotation. Patients had


Surgical procedures performed in the hygienic (1), difficulties with oral hygiene and pain during
functional (2), and mixed (3) groups of patients dressing maneuvers and had difficulties during
Group 1 Group 2 Group 3 sleep because of pressure of the internally rotated
Procedure Hygienic Functional Mixed Total spastic limb on the abdomen (Fig. 2).
Of the 16 patients undergoing surgery of the
PMr-t 15 7 7 29
LDr-t 5 1 6
shoulder, the preoperative degree of passive
TMr-t 6 1 7 abduction was 75 , with a median score on the
Adr 2 1 3 Ashworth scale for spasticity of the adductor
MDr 1 1 muscles of the shoulder of 3. Limitation of
SHB and 7 4 4 15 external rotation of the shoulder was on average
CBr-t 22 . Surgical intervention led to a mean abduction
Bt-frlnt 14 5 5 24 of the shoulder of 120.4 , with a passive external
BCr-t 9 3 3 15 rotation of 55 (P < 0.01).
BRr 14 4 4 22 Of the 23 patients surgically treated for
MEMr-br 9 6 6 21
personal hygiene outcomes, 10 patients reported
640 A. Landi et al / Hand Clin 19 (2003) 631–648

pain during personal hygiene, with the main The Lindon scale in patients with functional
problems related to difficulties in personal hygiene objectives ranged from 4–6 (mean 5.4), whereas
and dressing. All patients who reported pain the mean Pinzur [18] score was 1. Five patients
obtained resolution during daily hygiene man- had surgery limited to the elbow, one to the elbow
euvers following surgery. and shoulder, five to the elbow and forearm, and
All patients receiving surgery of the shoulder four to the shoulder, elbow, and forearm. Patients
for problems of personal hygiene and posture had with surgery to the elbow and shoulder district
a characteristic activation on the dynamic EMG presented problems with flexion and abduction of
of the anterior, middle, and posterior deltoid the shoulder and extension of the elbow for
muscles, pectoralis major, latissimus dorsi, teres reaching objects placed anteriorly or laterally.
major, and supraspinatus, ranging from III B All patients who had surgery to the elbow,
(prolonged phasic stage) to IV(continuous activa- using the functional objectives, showed a qualita-
tion) and V (spastic without voluntary control). tively normal dynamic EMG activation pattern
Muscles with the greatest involvement were the preoperatively in the three heads of the triceps.
pectoralis major, deltoid, latissimus dorsi, and The flexor muscles of the elbow present a type
teres major. IIIA or IIIB activation pattern (prolonged acti-
Of the 21 patients who had surgery to the vation for a brief period in successive phases),
elbow, all patients presented with a flexed elbow, with similar behavior in the brachioradialis,
difficulty in extension, and difficulties with dress- biceps, and brachialis muscles.
ing and maintaining adequate hygiene of the The surgical procedures performed in the
elbow fold. Functional Group are documented in Table 5.
The flexor muscles of the elbow studied Of these 16 subjects, 4 did not achieve functional
(biceps, brachialis, and brachioradialis) presented benefits on the Lindon scale (25%), and 2 (12.5%)
an activation ranging from IIIB (prolonged phasic showed an improvement of two ranks on this
phase) and VI (absence of activity). The biceps scale. The remaining patients improved by one
presented with more frequent activity of type IIIB level (62.5%). At statistical analysis with the
or IIIC (phasic with continuous activity), whereas Wilcoxon test for paired data, the improvement
the brachioradialis more frequently had level IV on the Lindon scale reached statistical significance
activation (spastic and loss of voluntary control). at a level of P < 0.01.
The performance of the brachialis was more often At the modified Sollerman scale, the mean
similar to that of the biceps, and in six patients did increment in upper arm function reached 3 points
not show any activity (grade VI). The biceps had (from 11 to 14.3 points) (P < 0.05).
more frequent activation in the context of In the single hand (dexterity) test of Jebsen, the
associated reaction to minimum efforts, whereas score increased on average by one item. Only four
the brachioradialis had a greater spastic activity to patients with surgery targeting improved func-
passive mobilization. Passive elbow extension tional objectives did not improve on the Jebsen
increased from 52.3 to 20.2 . Accordingly, the scale, even though one of these patients had
passive range of motion of the elbow increased a better performance on the bimanual testing.
from 12% to 73% of the complete range, on No significant differences were observed in the
average 27.8%. At the t-test for paired data, the Jebsen single hand instrumental scale in compar-
improvement reached significance at a level of ison with the Wilcoxon test for paired data,
P < 0.01. whereas a weak significance for the bimanual test
per paired data was found.

Group 2: surgery for functional outcome


Group 3: surgery for mixed problems
Preoperatively the 16 patients receiving surgery
for functional target had a mean score of 11 on Eleven subjects underwent surgery for a mixed
the Sollerman scale for bimanual or single hand target in which improvement was tied in part to
activities. personal hygiene and to a modest increase in bi-
The mean score following the performed tests manual activity, for the most part passive (Table 1).
of the Jebsen scale was 2, whereas on bimanual Of these patients, four had a true improvement
tasks the mean ranged from 2–3 (2.5) out of the of bimanual functioning at the Lindon scale,
five tasks proposed. whereas all patients had better range of motion
A. Landi et al / Hand Clin 19 (2003) 631–648 641

Fig. 2. (A,B) Left spastic hemiplegia following rupture of a cerebral aneurism. The main symptom was overnight undue
pressure by the spastic limb on the abdomen. (C,D) Follow-up following PMt, Bbr, ADr, MEMr, and BRdr. Posture
and speed on walking were affected positively and the pressure on the abdomen was relieved completely.

and higher scores on the items assessing disability neonatal resuscitation facilities. CVA and TBI are
and caregiver perception. now more common causes of spasticity.
In close relationship with the etiology, the
Discussion and conclusions average age for upper limb surgery has increased
consistently. Of the head-injured patients in the
The etiologic factors underlying spastic disor- present series, 19 recovered from coma and 12 out
ders of the upper limb have altered in the last of 50 had a CVA. The authors therefore tend to
century. Cerebral palsy from perinatal affections operate when consensus has been achieved be-
has decreased because of the improvement of tween the medical team, patient, and family, with
642

Table 5
Surgical procedures performed in the functional group (2) including the ones on joints, nerves, and tendons
Patient Age Sex Etiology Diagnosis Segment Site Procedures Joint Nerve Tendon transfers
FL 56 M C.V.A. Hemi S R PMr,CBr,SHBr — —
OG 25 F C.P. Tetra S+E+ F L PMr,ADr,Bzlnt,FCRt — —
De D 26 M T.B.I. Hemi S+E+ F R PMr,SHBr,CBr,LDr,TMr,MEMbr — —
VG 52 F C.V.A. Hemi S+E+ F L PMr,SHBr,CBr,MEMbr — —
BA 18 M C.P. Hemi S+E+ F L PMr,SHBr,CBr,BRdr,MEMr,PRC PRC — —
VF 37 M T.B.I. Hemi S+E R PMr,Er Er — —
PG 33 M T.B.I. Hemi S+F L PMr,FCUr,FCRr,PINd PINd —
MA 22 M T.B.I. Hemi E L Bzlnt,BCt,Er,UNant,HOr Er, HOr UNant —
MM 34 M T.B.I. Tetra E L Br,BRpr,Er Er —
T.R 27 F T.B.I. Hemi E R Er,UNant Er UNant —
PF 22 M T.B.I. Hemi E R Bltn,BCt,Er,HOr Er, HOr —
L 26 M T.B.I. Hemi E+F R MEMr,UNant,Er,HOr Er, HOr UNant —
G. S 27 M T.B.I. Tetra E+F L BRpr,FCUt,FCRt,PRC PRC — —
RC 29 M T.B.I. Tetra E+F R MEMbr,AbPLtoECRB — AbPLtoECRB
C.M 24 M C.V.A. Hemi E+F L MS,IMr,Grilli IMr — Grilli
Hemi Bzlnt,BCr,BRpr,MEMr,LEMr, Caps — FCRto ECRB
A. Landi et al / Hand Clin 19 (2003) 631–648

TD 20 M Enc. — E+F L FCRtoECRB,Caps —


10 cases 4 cases 3 cases
CP, cerebral palsy; CVA, cerebral vascular accident; TBI, traumatic brain injury; Enc, encephalitis; S, shoulder; E, elbow; F, forearm; Hemi, hemiplegia; Tetra, tetraplegia;
Hor, heterotopic ossificatio removal; PRC, proximal row carpectomy; IMr, interosseous membrane release.
A. Landi et al / Hand Clin 19 (2003) 631–648 643

the provision that the neurologic conditions are were able to ascertain and treat in two cases, one
stabilized. In seven cases of cerebral palsy, surgery of which belonged to the present series (patient
has been deferred in some instances to adulthood number 46).
when new functional requirements became appar- Surgery might carry undesired effects and
ent. Although surgery performed in adolescence complications, such as the negative impact of
carries a better prognosis [19], the authors agree scars on the psychology of the patient, especially
with Manske [20] that surgery also maintains its when the whole limb is affected, requiring ex-
efficacy in adulthood (Fig. 3). Many clinical tensive surgical procedures. The authors there-
pictures might be encountered when accurate fore have abandoned the deltopectoral incision
assessment of the entire upper limb is made. The and introduced the subpectoral approach (Fig. 3).
need to provide a simple chart that summarizes Probably in the future an endoscopic release
clearly the clinical picture at a glance has emerged might be used.
from the study of the authors’ case series. The When performing tenotomies of latissimus
modified Pinzur evaluation method [5,21] that dorsi and teres major, the possibility of creating
included the evaluation of the shoulder and elbow a cord effect at the axilla should be explained to
segments was considered in the end to be in- the patient (Fig. 4). This deformity is not
adequate. The authors now have adopted in all encountered after functional lengthening of lat-
neurorehabilitation centers of the Region of issimus dorsi and teres major. The range of
Emilia-Romagna a simple chart that offers at motion of the shoulder and elbow following
a glance a comprehensive picture of spasticity of surgery is improved significantly, as already
the upper limb and also provides a more objective observed in the literature [22]. In the authors’
method of recording data, which is a prerequisite series, improvement at the elbow was obtained in
for future outcome studies in this field (Boxes 1– extension, passive (in those patients surgically
3). Distinction is made between fixed postures treated for personal hygiene objectives) and active
(Box 1) and the residual active range of motion at for reach dexterity, as evaluated on dexterity tests
the shoulder and elbow. Assessment of the (tapping board performed on the vertical plane).
antagonistic muscles represents a crucial issue, as Undoubtedly, the muscle to be surgically treated
these can be potentiated. In the present classifica- must be chosen carefully. In particular, evaluation
tion, for example, the shoulder external rotators of the elbow is pivotal when deciding to intervene
might be present and can actively externally rotate on all three heads of the flexors or to limit surgery
the arm. Alternatively they might still be present to one of these.
and palpable but cannot be detected because of It is known that the brachioradialis muscle
the overwhelming activity of the internal rotators. often becomes dyssynergic [16,22]. In dynamic
Their presence or function can be identified easily EMG in this series, the authors observed that the
on dynamic EMG studies. For example, following brachioradialis most frequently had a spastic
release of the pectoralis major, coracobrachialis, activation profile during passive mobilization.
and subscapularis, a shoulder with no active The biceps and brachialis often maintained a cer-
external rotation before surgery might be tain degree of voluntary control that prolonged
upgraded to level 3 with the newly added possi- activation, usually in the opposite phase; these
bility of active external rotation in addition to the muscles often were involved in associated reaction
increased active range of motion. The same following minimal effort. Surgical intervention in
applies to active antero- and retroposition. this context aims to achieve significant objectives
Clinical examination of the elbow allows us to for the patient or caregiver. In some patients the
classify the patients in the group of fixed posture most important issue was flexion of the elbow
or within the group with an active range of during deambulation, which blocked posture or
motion. It is therefore possible to define the active transfers. In these situations, dynamic EMG
range of motion, to record the speed of move- evaluation of associated contractions, prevalently
ment, and to assess the capacity for active pro- of the biceps muscle, should orient the surgical
no-supination with the elbow in the flexed or approach toward a lengthening procedure of the
extended extreme activity range. Dynamic EMG biceps rather than release of the brachioradialis
studies are essential for understanding the degree muscle. In other instances in which difficulties in
of spasticity and dyssynergy related to each single dressing activities are recorded on the part of
muscle. Prono-supination might be impaired only caregivers, the hypertonic muscles evident on
by a dyssynergic anconeus muscle, as the authors passive mobilization should be addressed.
644 A. Landi et al / Hand Clin 19 (2003) 631–648

Fig. 3. (A) CP in premature birth. Clinical picture of spastic tetraparesis. (B) Surgical release of PM, B, BR, FCU, and
FCR. (C) The subpectoralis surgical approach allows proximal release of PM and short head of biceps and can be
concealed easily. (D,E) Surgery was performed with success at the age of 26 years for hygienic purposes.

All subjects undergoing interventions for particular, improvement was significant in tests
personal hygiene and posture defects reached the measuring caregiver workload in assisting the
established surgical objectives. These objectives patient, whereas specific and global disability tests
prevalently focused on hygiene of the armpit, pain were not modified significantly. This is not
control during mobilization of the elbow and surprising because many of these scales are not
shoulder, and improved capacity for dressing. In sensitive to the real impact of spastic hypertonus

Box 1. Box 2.
Classification of spastic disorders at the Shoulder functional assessment based on
shoulder and elbow: the fixed the active range of abduction from an
postures. internally rotated posture.
A. Landi et al / Hand Clin 19 (2003) 631–648 645

explanation lies in the loss of biomechanical


Box 3. advantage introduced after muscle lengthening.
In this case after surgical repositioning, the muscle
Elbow
a,b does not exert sufficient force on the joint be-
c cause the system of levers becomes less powerful.
a,b Indeed, surgery targeting personal hygiene aims to
a,b weaken the muscle by means of a substantial
d reduction of muscle mass, repositioning of the
a,b mechanical levers of action, detensioning of
a,b stretching receptors of the muscle, or lengthening
c of the muscle in case of retraction.
d In the interventions by the authors’ team for
a,b personal hygiene, the authors observed a remark-
able decrease of muscle activation during passive
stretching and these modifications remained at
follow-up over time.
and most patients submitting to surgery required Of the 16 patients treated for functional
a high degree of caregiver assistance. Patients with outcomes, the global level of disability on the
the most severe disabilities only showed improve- DRS was 6.2, equivalent to a moderate to severe
ment in the scales assessing caregiver burden and level; in patients with head trauma the cognitive
nonsignificant changes in scales evaluating dis- level was on average 7 (appropriate–automatic).
ability; this relates to the high degree of motor Even in this group of patients the disability scales
impairments (mean DRS of 11.7, equivalent to did not improve significantly after functional
severe disability), requiring a great deal of surgery. Concurrently, an increment in dexterity
assistance from caregivers for activities of daily on bimanual movements and on rapid movements
living. Moreover, subjects with head trauma who of the elbow in flexion–extension on the tapping
underwent surgery for personal hygiene objectives board was observed.
had a level of cognitive functioning of 5 on the In addition to a degree of improvement in
LCF scale (inappropriate–confused). dexterity on the Lindon scale, no significant
Surgery also improved range of motion of the improvements on the scales for specific or global
joints treated and permitted a significant reduc- disability were observed.
tion of spastic hypertonus as measured by the This may be explained by a low sensitivity of
Ashworth scale. The reduced hypertonus and the global disability scale. Another possibility is
improved range of motion can be explained by that many patients presented a stable clinical pic-
the lower traction of the myotactic spindle ture and a consolidated functional behavior that is
consequent on muscle lengthening. A possible difficult to modify. Significant improvement in the

Fig. 4. (A,B) TBI leading to a severe spastic tetraplegia with significant cognitive and behavioral disturbances. For
hygienic purpose an extensive shoulder release was performed (PMt, Bbr, BCr, LDt, TMt, BRpr, and MEMbr). The
cordlike effect caused by the myodesis effect by the retracted LD and TM is shown clearly on the lower border of the
axilla.
646 A. Landi et al / Hand Clin 19 (2003) 631–648

number of activities performed on single hand and The hemiparetic side generally showed negative
bimanual items of the Jebsen test was not signs such as weakness and insufficient muscle
detected, whereas the number of bimanual tasks recruitment as the principal problem, whereas
performed increased significantly. The better posi- impairment caused by spastic hypertonus and
tion of the shoulder and elbow may play an imp- retraction contributes only in part to the disability.
ortant role in these improvements (Fig. 5). The For the most part subjects tend to use the plegic
improvement of some bimanual functions, how- limb only for support. Releasing procedures on
ever, may not have an impact on the patient’s joints had a positive influence on the final range of
degree of disability. motion. Tendon transfers confined to a functional
Patients thus have an improved capacity to use group and decompressive procedures on the
both hands in activities requiring dexterity that, affected peripheral nerves improved the final
however, need not translate into a greater level of outcome. Significant improvement in posture and
autonomy in activities of daily living. Manual walking was obtained in two patients from Group
dexterity in tasks for the instrumental activities of 1, two patients from Group 2, and five patients
daily living (IADL) improved in some patients, from Group 3. The outcome measurements
such as use of utensils and computer keyboard selected were not sufficiently sensitive to detect
and capacity to grasp a bicycle handlebar. Para- the numerous but sparse improvements achieved
doxically, some patients report improved trans- in individual patients, who nonetheless considered
fers from sitting to standing position and better surgery rewarding. Though outcomes exceeded
deambulation. patient expectations in 10 cases, the authors had
A limited number of patients (three) had an to face a deluded patient and family in two cases.
appreciable increment of single hand and bi- Patients did not always have a clear idea of the
manual activities. objectives to be reached by surgery, and the

Fig. 5. (A) TBI in a sportsman. Concomitant compression of the PIN. (B) PMr, FCUr, and FCRr were performed in
conjunction with decompression on the posterior interosseous nerve because of a hypertrophic supinator brevis. (C) The
subpectoralis approach is easily conceivable. (D) Return of PIN function. (E) Good outcome with significant gain in
shoulder abduction. The patient now can swim the breaststroke and practice boxing at the gymnasium better.
A. Landi et al / Hand Clin 19 (2003) 631–648 647

in line with the realistic possibilities and effective


disabilities. Moreover, in the final evaluation this
test helps quantify the level of satisfaction and
capacity of performance perceived by the patient
in surgery for personal hygiene and aesthetics and
for recovery of gestures.
The literature does not present case series
reporting this type of surgery. The absence of
recurrence in the authors’ series makes surgical
treatment of the shoulder and elbow an interesting
approach as compared with other methods that
have a high recurrence, such as hyponeurotization
or the use of botulinum toxin.
In particular, the use of botulinum toxin for
segmental hypertonus should be limited to diag-
nostic use and to the early phases of disease,
whereas surgery should be proposed in later
stages, after the possible objectives to be reached
have been elucidated and other more conservative
methods exhausted.
The proposal for surgical treatment in any case
must be associated with an adequate program of
motor rehabilitative and occupational therapy.
Functional improvements are not possible with-
out an appropriate period of restorative care in
the postoperative period. This explains in part the
results obtained in the authors’ series in which
Fig. 5 (continued) improvements in personal hygiene and posture
were evident, whereas functional objectives had
a less predictable, though generally appreciable,
medical team was not always able to appreciate outcome.
the true expectations of the patient. Although Finally, when spasticity of the upper arm is
some rating scales used furnished a measure of the managed with a global approach and objectives
improvement of specific gestures, they do not are defined clearly in advance with the patient and
provide a comprehensive evaluation of the gain in caregivers, treatment of shoulder and elbow de-
autonomy. With these considerations in mind, the formities can achieve important results for per-
authors added two tests to the assessment battery sonal hygiene. When functional objectives are
to identify objective gains in autonomy and targeted, surgery at these proximal sites is a critical
patient expectations and to integrate the poor step that provides a releasing effect on the distal
sensitivity of the other tests. The two tests chosen joints [5,21] and prepares the upper limb for pos-
for their simple administration and efficacy were sible functional interventions at the forearm and
the Katz index and the COPM [10,23,24]. The hand.
former is a standardized test that evaluates the
capacity for self care in basic activities of daily
living (six hierarchically-ordered activities re- References
quired for autonomous living), with the aim of
documenting the level of independence before and [1] Thilmann AF, Burke DJ, Ryme WZ, editors.
after surgery. These data are used to classify the Spasticity mechanism and management. Berlin:
Springer-Verlag; 1993. p. 35–42.
patients based on the level of independence. The
[2] Herman R. The myotatic reflex. Clinico-physiolog-
shift from one class to a higher level permits ical aspects of spasticity and contracture. Brain
measurement of the efficacy of the intervention, 1970;93(2):273–312.
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The second test fulfills the initial need to better surgery. 2nd edition. Philadelphia: Lippincott; 1979.
understand patient objectives that often are not p. 263–83.
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SICM, editors. La patologia traumatica del gomito. [15] Bohannon RW, Smith MB. Interrater reliability of
Parma: Mattioli; 1998. p. 80–90. a modified Ashworth scale of muscle spasticity.
[5] Landi A, Caserta G, Leti Acciaro A, Della Rosa N, Phys Ther 1987;67(2):206–7.
Gagliano MC. Il trattamento della spasticità [16] Keenan MA, Haider TT, Stone LR. Dynamic
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Chir Mano 2002;39(1):3–14. Surg 1990;15A(4):607–14.
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adult. Clin Orthop 1982;168:38–41. bral palsy surgery of the upper extremity in
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superiore. Milan: Edizioni Centro Studi Mano; [20] Manske PR. Cerebral palsy of the upper extremity.
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[10] Law M, Baptiste S, Pollock N, Carswell A, McColl [21] Landi A, Mulcahey MJ, Caserta G, Della Rosa N.
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JM. Quantifying associated reactions in the paretic adjustment and social behaviour in the community:
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Hand Clin 19 (2003) 649–655

Surgical management of forearm pronation


Claudia R. Gschwind, MD, FMH, FRACS
Department of Hand Surgery and Peripheral Nerve Surgery, Royal North Shore Hospital,
St. Leonard NSW, 2065 Sydney, Australia

Pronation contracture of the forearm is part of [6]. All groups had significant improvement of
the common pattern of upper limb deformity in forearm supination postoperatively.
cerebral palsy. It is caused by a muscle imbalance In Group 1 with active supination beyond
between supinatory and pronatory muscles. Spas- neutral, no specific procedure is indicated to
ticity in pronator teres, pronator quadratus, and improve supination.
the whole flexor group can mask coexisting supi- In Group 2 with active supination up to or less
nation activity. than neutral, a release of spastic muscles with
To separate elbow, wrist, finger, thumb, and pronatory activity should improve supination.
pronation deformity into separate groups seems The degree of active supination and the residual
artificial. It is, however, didactically helpful and in contracture in the flexor pronator group deter-
clinical examination improves the understanding mine the range of active forearm rotation
of the dynamics of deformity. Understanding achieved in this group.
muscle imbalance is necessary when devising a In clinical examination, Groups 3 and 4 show
treatment plan. Important points of preoperative no active supination. In Group 3, passive supina-
assessment (eg, general neurologic condition, type tion is easily possible, and the main problem,
of neuromuscular disorder, extent of limb in- therefore, is the lack of an active supinator. This
volvement, age, and volitional control over limbs) deficit can be corrected by a pronator teres trans-
have been outlined by Zancolli [1] and others [2,3]. fer or even a flexor carpi ulnaris (FCU) transfer.
To select suitable candidates for surgery, repeated In Group 4, a release of spastic muscles with
clinical examination is necessary. The active and pronatory effect is indicated to allow possible ac-
passive range of joint motion, hand sensibility, tive supination to be unmasked. If after a release
and the functional level of the limb are noted. The there is no active supination present, a pronator
principle of surgery is to decrease tone in over- teres transfer may be contemplated (Box 3).
active muscle groups with release procedures and In children with severe hyperpronation, radial
to augment weak muscles by tendon transfers. head dislocations have been described [7]. The re-
Joint or soft tissue contractures have to be commended treatment is pronator teres rerouting,
released first to allow passive motion. Only if rather than excision of the head, which leads to an
joints and ligaments permit motion can muscle increase of the deformity. Radial osteotomy also
releases and tendon transfers be successful. has been advocated to improve forearm position
Effective treatment of elbow, wrist, and finger in severe cases. Inferior radioulnar joint fusion
flexion deformity alter the balance between mus- may be an option if the pronatory forces cannot
cles involved in pronation and supination of the be overcome by soft tissue procedures or in athe-
forearm. Box 1 lists all procedures improving toid disorders in which tone changes unpredict-
supination (Fig. 1). Several different classifications ably. This fusion is performed in slight supination
of wrist and finger deformity have been proposed with the radial head in a reduced position [8].
in the literature [2–5]. A classification for prona- The worst complication of surgery for pro-
tion deformity, a proposed treatment plan, and nation deformity is to create a supination de-
results of surgery were published in 1992 (Box 2) formity. The author therefore cautions against
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00030-1
650 C.R. Gschwind / Hand Clin 19 (2003) 649–655

Box 1. Procedures altering balance in favor of forearm supination

Releases
Pronator teres release
Pronator quadratus release
Flexor pronator slide
Flexor aponeurotic release
Fractional lengthening of flexor tendons
Flexor carpi ulnaris tenotomy
Transfers
Pronator teres rerouting
Flexor carpi ulnaris to extensor carpi brevis transfer
Flexor carpi ulnaris to extensor digitorum communis transfer
Extensor carpi ulnaris to extensor carpi radialis brevis transfer
Flexor profundus to flexor superficialis transfer

releasing pronator teres distally at its insertion the brachioradialis musculotendinous junction.
and pronator quadratus at the same time. The In the subcutaneous layer, the lateral cutaneous
danger of creating a supination deformity is less nerve of the forearm and the superficial branch
pronounced if pronator teres is only partially of the radial nerve are preserved. Between the
released, as in a proximal flexor aponeurotic brachioradialis tendon and the wrist extensors,
release, but is certainly present when the release the insertion of pronator teres is identified and the
is distal at its insertion or massive, as in a flexor tendon is transversely divided. Some investigators
pronator slide. Supination deformity is a function- suggest excision of some of the tendon [10]
al disadvantage to chair-bound patients [9]. (Fig. 3).
Typical activities in the wheelchair, like typing,
grasping and releasing, and pointing are easier in Pronator quadratus release
pronation than supination (Fig. 2). Electrophysiologic studies on volunteers by
Basmajian and Delucca found pronator quad-
Surgical procedures for pronation deformity ratus to be the prime pronating muscle in the
forearm. This was irrespective of angulation of the
Releases elbow joint or position of the forearm in space
Pronator teres release [11]. No studies have been performed to clarify if
Surgical technique. A longitudinal 3-cm incision is this is similar in patients with a spasticity disorder.
made over the middle third of the radius, dorsal to The pronator quadratus release rarely is men-
tioned in the literature [3,5,12,13]. After an ag-
gressive flexor pronator slide or a distal pronator
teres release, pronator quadratus remains as the
only pronator. If it is under poor volitional con-
trol, pronation may be inadequately initiated or
maintained. This may result in a supination de-
formity, particularly if at the same time an FCU
to extensor carpi radialis brevis (ECRB) or exten-
sor digitorum communis (EDC) transfer for wrist/
finger flexion deformity is performed. In such
a case the author recommends that pronator
quadratus not be released.
Surgical technique. A 4-cm longitudinal incision is
Fig. 1. Extensor carpi ulnaris to extensor carpi radialis placed radial to the radial artery in the distal
longus for rebalancing of the wrist in extension also aspect of the forearm. Care is taken to cauterize
produces a supinatory effect. small branches of the radial artery. The pronator
C.R. Gschwind / Hand Clin 19 (2003) 649–655 651

Box 2. Classification of pronation deformity

Group 1 Active supination beyond neutral


Group 2 Active supination to or less than neutral
Group 3 No active supination: free passive supination
Group 4 No active supination: tight passive supination

quadratus is detached from its insertion into the median and ulnar nerves are identified and
radius, and with an elevator is detached all across protected (Fig. 5). The more radical distal FAR
and from the interosseous membrane (Fig. 4). is performed through a transverse incision 9–12
cm distal to the medial epicondyle.
Flexor aponeurotic release
The flexor aponeurotic release (FAR) was
described by Zancolli and is suitable in children Flexor pronator slide
only. In adults there is usually a component of This procedure, first described by Page, ad-
myostatic contracture present, and a flexor pro- dresses several problems [14]. The release of the
nator slide or fractional lengthening of tendons is flexor pronator mass from the epicondyle im-
recommended instead. FAR allows muscle fibers proves flexion contractures of the elbow and of
to be stretched after release of all fascial en- the wrist and fingers (Fig. 6). Detachment of the
velopment but does not tenotomize any tendons pronator teres origin decreases the pronatory
extramuscularly. A ‘‘distal FAR’’ divides not only effect of pronator teres in the forearm. This
the fascia and septa but also the intramuscular procedure often is performed in conjunction with
tendon, as in fractional lengthening [8]. If in releases/lengthening of biceps, brachialis, and bra-
Group 4 a ‘‘distal FAR’’ is performed at the same chioradialis to improve elbow contractures. The
time as an FCU to ECRB or to the finger extensor flexor pronator slide is an extensive procedure and
and is used to improve wrist and finger extension, may lead to substantial weakening of the finger
the author does not recommend a release of flexors [8,15]. Some investigators believe it should
pronator quadratus at the same time, or a supina- be performed only if finger flexion causes a hygiene
tion deformity may develop. Because pronator problem and not in the functional hand [4].
teres has been divided distally, the only option for Recently, selective release of the flexor origin in
correcting the supination deformity is to then conjunction with FCU transfer was described [16].
perform a biceps rerouting [6] (see Fig. 2).
Surgical technique. A lazy S or a straight incision
Surgical technique. A 5-cm transverse incision is is made, starting 5 cm proximal to the medial
placed in the proximal forearm, 6 cm distal to the epicondyle and extending to the proximal third of
medial epicondyle. In the subcutaneous layer, the the forearm. The ulnar nerve, median nerve, and
medial cutaneous nerve of the arm and the larger brachial artery are identified and preserved. The
veins are protected. A strip of 2 cm of forearm flexor pronator mass is detached from the medial
fascia is excised transversely over the flexor epicondyle, the proximal ulna, and the interos-
pronator mass. All fascial septa down to the ulna seous membrane. The slide allows for 4–6 cm of
and interosseous membrane are divided. The lengthening (Fig. 6).

Box 3. Recommended procedures for pronation deformity

Group 1 No specific surgery indicated


Group 2 Pronator quadratus release ± flexor aponeurotic release
Group 3 Pronator teres transfer
Group 4 Pronator quadratus release + flexor aponeurotic release
652 C.R. Gschwind / Hand Clin 19 (2003) 649–655

Fig. 2. Postoperative supination deformity on the left Fig. 4. Pronator quadratus has been detached from the
after release of pronator quadratus, distal flexor radius and interosseous membrane.
aponeurotic release, proximal row carpectomy, and
flexor carpi ulnaris to extensor carpi radialis brevis on the radius [17]. In the original description, the
transfer. This complication required correction by a bi- tendon is detached with a strip of periosteum and
ceps rerouting procedure.
rerouted through a bony canal in the radius. In
two patients in his series of 22 cases, a fracture of
the radius occurred [18]. A simplification of the
Tendon transfers
procedure in analogy to Zancolli’s biceps rerout-
Pronator teres rerouting ing procedure has since been described [6].
This procedure, described by Sakellarides,
Surgical technique. Through a longitudinal in-
reverses the rotational action of pronator teres
cision dorsal to the brachioradialis musculotendi-
nous junction in the mid third of the radius, the
pronator insertion is identified. The tendon is
tenotomized in a Z-fashion whereby the proximal
limb of the Z is lengthened with a periosteal strip.
The distal Z limb is rerouted around the radius to
create a supinatory effect (Figs. 7 and 8).

Fig. 5. A 2-cm strip of fascia and all septa has been


removed. The median nerve is preserved. The tendon of
pronator teres is preserved in this flexor aponeurotic
Fig. 3. Pronator teres release with or without excision of release. In the distal, more aggressive release, the tendon
tendon. would be divided.
C.R. Gschwind / Hand Clin 19 (2003) 649–655 653

Fig. 6. The muscles of the flexor pronator mass are


detached from the medial epicondyle and the ulna. This
allows a slide of 4–6 cm.

Fig. 7. Pronator teres rerouting. (A) The tendon is


divided at its insertion and may be elongated with a strip
of periosteum. A drill hole is placed at the site of
insertion. (B) The tendon has been rerouted and is fixed
with sutures into the drill hole to create a supinatory
effect on the radius.

Fig. 9. The flexor carpi ulnaris to extensor carpi brevis


transfer around the ulnar border of the forearm
produces a supinatory effect.

Flexor carpi ulnaris transfer


Rerouting of FCU around the ulnar border of
the wrist into the short radial wrist extensor
Fig. 8. Modified pronator teres rerouting procedure. (A) (Green transfer) or into the EDC tendons will, by
The tendon is tenotomized in a Z fashion. (B) Routing of its due course, have a supinatory effect on the
the tendon insertion reverses pronatory action of pro- forearm [19,20]. The surgical technique is de-
nator teres. scribed elsewhere (Fig. 9).
654 C.R. Gschwind / Hand Clin 19 (2003) 649–655

Postoperative treatment References


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therefore have to be viewed in the context of
hand surgery. 2nd edition. Philadelphia: Lippincott;
surgery for elbow, wrist, and finger flexion 1968. p. 263–83.
deformity. If a patient in Group 1 (in whom no [6] Gschwind C, Tonkin M. Surgery for cerebral palsy:
specific surgery is indicated to improve supina- part 1. Classification and operative procedures for
tion) undergoes a FAR for wrist and finger flexion pronation deformity. J Hand Surg 1992;17B(4):
deformity, supination improves by 20 –30 [6]. 391–5.
One can expect similarly approximately 20 of [7] Pletcher DF, Hoffer MM, Koffman DM. Non-
increased supination if FCU is transferred around traumatic dislocation of the radial head in cerebral
the ulnar border into ECRB to improve wrist palsy. J Bone Joint Surg 1976;58A(1):104–5.
extension [21]. If the Green transfer is performed [8] Tonkin MA. The upper limb in cerebral palsy.
Current Orthop 1995;9:149–55.
on a patient in Group 3, a pronator teres re-
[9] Zancolli EA, Goldner LJ, Swanson AB. Surgery of
routing may not be necessary or may be per- the spastic hand in cerebral palsy: report of the
formed secondarily if supination is inadequate. Committee on Spastic Hand Evaluation (Interna-
In 1988, Strecker et al compared results of tional Federation of Societies for Surgery of the
pronator tenotomy with pronator rerouting and Hand). J Hand Surg 1983;8(5 part 2):766–72.
found a greater increase of supination after [10] Gelberman RH. Cerebral palsy. In: Gelberman RH,
pronator teres rerouting than after tenotomy editor. Operative nerve repair and reconstruction.
[22]. Their preoperative assessment did not reveal Philadelphia: Lippincott; 1991. p. 1455–75.
details of passive preoperative supination. Per- [11] Basmajian JV, De Luca CJ. Muscles alive, their
haps rerouting of pronator teres alone, in the functions revealed by electromyography. 5th edition.
Baltimore: Williams and Wilkins; 1985. p. 561.
presence of contractures of all flexors and tight
[12] Pollock GA. Surgical treatment of cerebral palsy.
passive supination, would not result in improved J Bone Joint Surg 1962;44B:68–81.
supination unless these muscles are released. [13] Samilson RL, Morris JM. Surgical improvement of
the cerebral-palsied upper limb. J Bone Joint Surg
1964;46A(6):1203–16.
Summary [14] Page CM. An operation for the relief of flexion
contracture in the forearm. J Bone Joint Surg
Active volitional supination enhances upper 1923;5:233–4.
limb function. In the spastic forearm with pro- [15] Hoffer MM. The use of pathokinesiology laborato-
nation deformity, careful preoperative assessment ry to select muscles for tendon transfers in the
of muscle function is necessary to design a treat- cerebral palsy hand. Clin Orthop 1993;288:135–8.
ment plan to improve supination. Concomitant [16] El-Said NS. Selective release of the flexor origin
with transfer of flexor carpi ulnaris in cerebral
procedures performed for flexion contractures
palsy. J Bone Joint Surg 2001;83B(2):259–62.
have to be taken into consideration. The release [17] Sakellarides HT, Mital MA, Lenzi WD. The
of the forearm, wrist, and fingers from a con- treatment of pronation contractures of the forearm
tracted pronated position may unmask active in cerebral palsy. J Hand Surg 1976;1(1):79–80.
supination or may make the need for a tendon [18] Sakellarides HT, Mital MA, Lenzi WD. Treatment
transfer with supinatory activity obvious. of pronation contractures of the forearm in
C.R. Gschwind / Hand Clin 19 (2003) 649–655 655

cerebral palsy by changing the insertion of the [21] Beach WR, Strecker WB, Coe J, Manske PR,
pronator radii teres. J Bone Joint Surg 1981; Schoenecker PL, Dailey L. Use of the Green transfer
63A(4):645–51. in treatment of patients with spastic cerebral
[19] Green WT. Tendon transplantation of the flexor palsy: 17-year experience. J Pediatr Orthop 1991;11:
carpi ulnaris for pronation-flexion deformity of the 731–6.
wrist. Surg Gynecol Obstet 1942;75:337–42. [22] Strecker WB, Emanuel JP, Dailey L, Manske PR.
[20] Green WT, Banks HH. Flexor carpi ulnaris trans- Comparison of pronator tenotomy and pronator
plant and its use in cerebral palsy. J Bone Joint Surg rerouting in children with spastic cerebral palsy.
1962;44A:1343–52. J Hand Surg 1988;13A(4):540–3.
Hand Clin 19 (2003) 657–665

Surgical management of wrist and finger deformity


Ann E. Van Heest, MD
University of Minnesota, Department of Orthopedic Surgery, Hand Surgery Section,
Gillette Children’s Specialtycare, Shriner’s Hospital–Twin Cities Unit, 2450 Riverside Avenue South,
R200, Minneapolis, MN 55454, USA

The major goal in surgical reconstruction of problem of inadequate release is as much of a


the wrist and fingers is to restore muscle force functional concern as inadequate grasp strength.
balance across the joints to improve function and Finger and wrist deformity must be addressed to
provide a less ‘‘palsied’’ appearance. Treatment provide overall grasp and release function.
is aimed at altering the imbalance between the
spastic flexor-pronator muscle group and the
paretic extensor–supinator group. Additionally, Wrist treatment options
surgical treatment targets treating joint imbalance
to prevent fixed deformity. The most common deformity of the wrist is
The surgeon is required to assess carefully the flexion, usually with ulnar deviation. This is
type of deformity and its treatment at each upper probably the most functionally disabling defor-
extremity joint separately and then combine them mity of the upper limb in cerebral palsy, as it
to organize a comprehensive surgical reconstruc- significantly interferes with grasp and release
tive plan. Adequate shoulder, elbow, and forearm function. The wrist flexion deformity is often the
function is necessary for the patient to be able to most obvious physical manifestation of the pa-
position the limb in space appropriately; adequate tient’s cerebral palsy and correction of the ‘‘palsy’’
wrist, finger, and thumb function is necessary for position helps with the patient’s self esteem.
appropriate grasp, pinch, and release. Because the Several different surgical options exist; the
extrinsic finger muscles are biarticular (ie, they choice depends on the degree of deformity and
cross the wrist and finger joints), the position of the extent of volitional control of each muscle
the wrist significantly affects the position and involved. Application of the following surgical
function of the finger extrinsic muscles. The wrist principles is a necessary part of the art of designing
and fingers thus commonly are assessed together. a successful reconstructive plan: release or length-
Evaluation of wrist and finger deformity most ening the deforming spastic muscles (flexor carpi
commonly has followed Zancolli’s classification ulnaris [FCU], flexor carpi radialis [FCR]), transfer
[1] as shown in Box 1. of tendons to augment the weak antagonist muscles
A normal pattern of grasp and release is (extensor carpi radialis longus/brevis [ECRL/B]),
necessary for hand function. When the wrist is and stabilization of the joint only for the severe,
in substantial flexion, grasp strength is decreased fixed, nonfunctioning wrist (wrist fusion). Surgical
as the finger flexors become relatively shortened. treatment options for correction of wrist and finger
If the wrist is corrected to neutral, manually or in deformities are outlined in Table 1.
a splint, finger flexion strength increases, but often
there is a loss of the ability to release. The Release/lengthening of spastic flexor muscles
If the wrist flexion deformity is mild and wrist
extensor control exists, weakening the wrist
flexors through surgical fractional lengthening
E-mail address: vanhe003@umn.edu or with a series of botulinum injections [2] in
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00076-3
658 A.E. Van Heest / Hand Clin 19 (2003) 657–665

Box 1. Zancolli classification [1]

Group 1. Active finger extension with less than 20 of wrist flexion
Group 2. Active finger extension with more than 20 of wrist flexion
Group 2a. Active wrist extension with the fingers flexed
Group 2b. No active wrist extension with the fingers flexed
Group 3. Wrist and finger extension absent even with full wrist flexion

combination with serial casting, splinting, or deformity, although this may require FCU length-
stretching may provide sufficiently increased wrist ening concomitantly, but has the disadvantage of
flexor muscle lengthening to decrease overall wrist not providing significantly more wrist extension
flexion posturing. If the mild wrist flexion de- force than is already present. Using the FCU
formity exhibits concomitant wrist ulnar devia- tendon has the advantage of removing its force as
tion, the FCU only would be lengthened. If a more a spastic wrist flexor/ulnar deviator while trans-
severe wrist flexion deformity exhibits concomi- ferring its forces into wrist extension, but has the
tant finger flexion and pronator spasticity, the disadvantage of overcorrection if the deformity is
entire flexor pronator mass can be lengthened not severe or if the transfer is sutured too tightly,
using a flexor pronator slide [3,4]. particularly in the younger child. FCU to ECRB
tendon transfer is demonstrated in Fig. 1.
The FCU to radial wrist extensor tendon
Tendon transfers to augment weak or absent
transfer was first described by Green [5]. Many
wrist extensors
studies have reported the functional improvement
If the wrist flexor deformity is more severe and seen by improved wrist function after the Green
wrist extensors are not functional, tendon trans- tendon transfer [6–9]. Wolf et al [10] reported 16
fer surgery to augment wrist extension may be children treated with FCU to ECRL tendon
necessary. Muscles that can be transferred into transfer with an average follow-up of 4 years
wrist extensors include the brachioradialis (BR), (range, 1–9 years). Average final resting position
the extensor carpi ulnaris (ECU), or the FCU. was 9 of extension with 14 of 16 patients reporting
Using the BR or the ECU as the donor tendon has improved function and 16 of 16 patients report-
the advantage of leaving both flexor tendons ing improved cosmesis. El-Said [11] has described
intact, thus avoiding overcorrection, but has the the use of a selective release of the flexor origin
disadvantage of not achieving balance unless the concomitantly with transfer of the FCU in 35
wrist flexors are lengthened if their spasticity is patients with an average follow-up of 4 years. The
significant. Using the ECU tendon has the appearance of the hand and forearm improved in
advantage of correction of the ulnar deviation all patients; all gained improved mobility of the

Table 1
Surgical treatment options
Wrist flexion ulnar deviation Finger deformities
Tendon Flexor pronator slide Flexor pronator slide
Release/Lengthenings FCR lengthening FDS lengthening
FCU lengthening FDP lengthening
Tendon transfers FCU to ECRB Superficialis to profundus transfer
FCU to EDC Lateral band rerouting (swan neck)
BR to ECRB
ECU to ECRB
Joint stabilization Wrist fusion PIP fusion
Proximal row carpectomy DIP fusion
BR, brachioradialis; DIP, distal interphalangeal joint; ECRB, extensor carpi radialis brevis; ECU, extensor carpi
ulnaris; FCR, flexor carpi radialis; FCU, flexor carpi ulnaris; PIP, proximal interphalangeal joint.
From Zancolli EA. Structural and dynamic bases of hand surgery. 2nd edition. Philadelphia: JB Lippincott; 1979;
with permission.
A.E. Van Heest / Hand Clin 19 (2003) 657–665 659

forearm, wrist, and hand. Long-term follow-up of a finger flexor lengthening will be necessary as
25 patients [7] treated at a mean age of 8 years with part of the procedure. This is a common combi-
a mean follow-up of 8 years, 7 months reported nation of procedures for many surgeons [18,19]. If
a final arc of motion of 44 of wrist extension and the patient does not have finger extensor control
19 of wrist flexion. Two patients required revision to allow for release of grasped objects, a transfer
for overcorrection into a wrist extension/supina- into the finger extensors (EDC) may be indicated.
tion deformity. They recommended FCU to radial Other series have found that transfer of the FCU
wrist extensor tendon transfer for patients who into the EDC is rarely necessary [8,20], as bal-
lack active wrist extension but who have good ancing the wrist may be the key component to
digital extension with the wrist passively extended unmasking finger function.
above neutral.
Newer concepts in determining optimal intra-
operative tension have been described recently.
Wrist stabilization
Lieber et al [12] reported the use of intraopera-
tive sarcomere length measurements and its rela- If the patient has a severe wrist joint contracture
tionship to tensioning of tendons during tendon limiting functional use of the hand (even as a
transfer surgery. Because it is not possible to paperweight), consideration should be given to
predict the relationship between passive tension a proximal row carpectomy (PRC) to shorten the
and muscle length, these investigators have advo- skeleton or a wrist fusion to hold the wrist in a fixed,
cated the experimental use of an intraoperative more functional position. The PRC is used in
device that allows measurement of sarcomere combination with tendon transfers and releases in
length as a basis for tensioning tendon transfers. patients in which passive extension of the wrist does
Although this new measurement modality has been not reach the neutral position. Passive mobility
shown to be significant in an experimental model of the wrist is a necessary prerequisite to tendon
[13–16], it has not been tested specifically in the transfer surgery, which aims to improve active
clinical setting. Whether transfers set at optimal mobility. Tonkin and Gschwind describe treatment
sarcomere length are associated with improved of 34 patients with wrist and finger flexion de-
functional outcomes remains to be studied. formities; four patients were treated with a combi-
Another concern regarding the FCU transfer nation of PRC, FCR, or FCU to ECRB, flexor
into the ECRB/L is the optimal vector for aponeurotic release, and thumb and pronation
placement (ie, the extent to which the FCU origin procedures. The postoperative extension was 8
should be released and the angle at which the and all regained some ability to extend the fingers.
tendon is inset). In the original description, Green Wrist fusion predictably maintains the wrist in
[5] recommended the FCU transfer be into the a fixed position and usually is indicated for
‘‘tendon of the ECRB to give more central action improved cosmesis and use of the hand as a
in dorsiflexion, whereas the tendon of the ECRL, paperweight in the skeletally mature individual
which is more radial in position, gives a better [21]. The proximal carpal row can be removed as
direction of pull for supinator action and gives part of the wrist fusion to facilitate positioning of
better correction of the ulnar deviation.’’ Cada- the wrist into slight extension [22], as shown in Fig.
veric studies subsequently have shown that in- 2. Hargreaves et al [23] reported 11 wrist fusions in
sertion into the ECRB versus the ECRL did not 10 patients treated at an average age of 18.8 years
change the supination effect of the transfer sig- (range, 13–35). Concomitant PRC was performed
nificantly. Rather, freeing the muscle up to the in eight wrists and soft tissue releases in three.
proximal one third of the forearm gave it a less Improved function from preoperative ‘‘none’’ or
acute angle of insertion and increased its supina- ‘‘assist’’ levels was improved postoperatively to
tion effect [17]. The FCR should not be used as ‘‘assist’’ to ‘‘simple’’ function. Fusion was achieved
a transfer for wrist extension, as it would provide in all cases, using plate fixation in nine patients and
a secondary pronation vector that would exacer- crossed K-wires (because of open physes) in two
bate the patient’s pronation deformity. patients. Wrist fusion is contraindicated in the
In all cases of transfer into the wrist extensors, individual who uses wrist flexion tenodesis for
the finger function must be assessed preopera- release function, as this function would be lost if
tively with the wrist in neutral, the desired the wrist were to be fixed in a single position. It
postoperative position. If the finger flexors are may be particularly beneficial when an athetoid
too tight when the wrist is brought into neutral, component dominates.
660 A.E. Van Heest / Hand Clin 19 (2003) 657–665
A.E. Van Heest / Hand Clin 19 (2003) 657–665 661

Finger surgery extrinsic flexors may unmask intrinsic spasticity


that may need to be addressed secondarily.
The most common finger deformities are the
In addition to release of the spastic flexors of
spastic flexion deformity and the swan neck
the fingers, the question may arise as to whether
deformity. Spastic flexion deformities causing
tendon transfers may be necessary to augment
a clenched fist need to be addressed in conjunc-
finger extension. Smith [27] recommends tendon
tion with the wrist flexion deformity. The flexor
transfers for finger extension only after release
digitorum superficialis (FDS) and flexor digito-
procedures are completed to the wrist and fingers.
rum profundus (FDP) muscles are biarticular,
Often it may take 6 months or longer until the tone
crossing the wrist and finger joints. They thus
of the digital extensors returns after the persistent
need to be lengthened in concert with the wrist
stretch of the extensors has been released by
flexion deformity correction as part of a flexor
lengthening of the flexors. Hoffer et al [28]
pronator slide or with selective fascial length-
reported 38 patients treated with tendon transfers
enings. The flexor pronator slide was described
to improve extension of the wrist and fingers.
originally by Page [24] in 1923, reporting on six
Twenty patients were treated with transfer of the
patients of whom two had cerebral palsy. Several
FCU to the ECRB and 18 patients were treated
subsequent reports [3,4] discuss the role of the
with transfer of the FCU into the EDC. Of these
flexor pronator slide in decreasing spastic forces in
18 patients, 16 showed significant functional
the wrist and finger flexors simultaneously and
improvement. He recommended that the FCU be
how this may unmask wrist and finger extension.
transferred to the EDC in patients with the
For the more severe clenched fist deformity,
apparent inability to release their hands (ie, cannot
cosmesis and hygiene may be the greater concern.
extend their digits with their wrist extended).
Superficialis to profundus (STP) transfer is used
Other series have found that transfer of the
to treat severe spastic flexion contractures of
FCU into the EDC is rarely necessary [8,20], as
the hand. It is the treatment of choice in the
balancing the wrist may be the key component to
nonfunctional hand with a spastic clenched fist
unmasking finger function.
deformity. Palma et al [25] have performed more
After clenched fist deformity, the next most
than 75 STP transfers to relieve pain, improve
common deformity seen is the swan neck. The
hygiene, and ease daily activities for patients and
pathophysiology of this deformity in cerebral
caregivers and found the procedure to be effective,
palsy is the dynamic imbalance of the muscles
predictable, and safe. It was combined commonly
acting on the proximal interphalangeal (PIP)
with a wrist arthrodesis, carpal tunnel release, and
joint, causing PIP joint hyperextension with distal
ulnar motor neurectomy. Braun et al [26] reported
interphalangeal joint flexion (Fig. 3). In cerebral
that the STP operation ‘‘attained the limited goals
palsy, swan neck deformities are caused by
set for this initial group of patients with severe
intrinsic muscle spasticity, often augmented by
flexion problems, who had little hope for restored
overactivity of the extrinsic finger extensors.
function of individual flexor tendons. In twenty
Spasticity of the intrinsic muscles causes overpull
one patients, the resting position of the hand was
of the lateral bands, accentuating PIP joint
improved.’’ They warn that release of the spastic

b
Fig. 1. (A) Preoperative view of dynamic wrist flexion deformity resulting in a poor active assist hand with significant
disability for grasp and pinch functions. This 16-year-old girl had normal intelligence, moderate sensibility (10 of 12
objects stereognosis function), and high motivation. No active wrist extension was present preoperatively, with good
control of active finger extension. She showed good preoperative selective control of the FCU despite its spasticity. (B)
Through a longitudinal incision extending the distal two thirds of the forearm along the ulnar border, the FCU was
harvested from its insertion. (C) A curvilinear incision over the radial wrist extensors was used for weaving the FCU into
the ECRB just proximal to the extensor retinaculum. The same incision was used as part of the EPL rerouting. A
separate incision was used in the antecubital fossa for a biceps lengthening and brachialis fascial release. (D) The FCU is
harvested off its pisiform insertion and freed from its fascial attachments. (E) The FCU tendon is transferred dorsally,
removing it as a wrist flexor and providing its force as an active wrist extensor. The tendon is tensioned so that the wrist
lies antigravity at rest in a neutral position. (F) Following the procedure, active wrist extension was achieved for effective
grasp. Finger function was good for release. Good active assist function was achieved.
662 A.E. Van Heest / Hand Clin 19 (2003) 657–665

Fig. 2. (A) Preoperative radiographs of a fixed flexion wrist contracture in a skeletally mature 17-year-old boy. Note the
finger extension present preoperatively, indicating minimal finger flexor contractures. (B) Proximal row carpectomy
combined with wrist fusion by plating was used. Adequate finger excursion was present so that concomitant finger flexor
lengthening was not necessary in this case.

extension. With chronic intrinsic spasticity, over- Finally, excessive lengthening or surgical release
pull of the lateral band causes incompetence of the of the FDS, such as used in the STP transfer, often
transverse retinacular ligament, stretching of the unmasks intrinsic spasticity, resulting in significant
PIP joint volar plate, and resultant dorsal sub- swan neck deformities, as the flexion forces on the
luxation of the lateral bands. Additionally, some PIP joint are diminished iatrogenically.
patients with cerebral palsy have better volitional The indication for surgical correction is lock-
control of their extrinsic finger extensors than they ing swan neck deformities (usually greater than
have of their wrist extensors. Patients thus at- 40 ) that are not responsive to splinting and that
tempt to extend their wrists through activation interfere with function.
of their extrinsic finger extensors, causing meta- For the patient with significant wrist flexion
carpophalangeal (MCP) joint extension and exac- deformities and only mild swan-necking, surgi-
erbating PIP joint hyperextension (swan-necking). cal correction of wrist position alone may be
A.E. Van Heest / Hand Clin 19 (2003) 657–665 663

Fig. 3. (A) Pathophysiology of swan neck deformity in cerebral palsy. In cerebral palsy, the intrinsic muscles are spastic.
The extrinsic finger extensors are overactive in their attempt to augment wrist extension when a wrist flexion deformity
exists. Intrinsic spasticity combined with extrinsic overpull causes PIP joint hyperextension with resultant incompetency
of the transverse retinacular ligament and volar plate. PIP joint hyperextension concentrates the extension forces at the
PIP joint with slackening of the terminal tendon allowing DIP joint flexion. (B) This patient exhibits swan neck
deformities that lock in extension and inhibit his abilities to grasp his walker.

adequate for treatment. For the patient with deficiency in conjunction with their motor de-
severe swan necking (>40 ), rebalancing of the ficiency [36,37], several recent studies have dis-
muscle forces at the PIP joint is necessary. proved the previous doctrine that hand surgery
Surgical options include lateral band rerouting should not be performed on children with
[29,30], lateral band tenodesis [31], superficialis sensibility deficiencies. The author’s report [20]
tenodesis [32], spiral oblique ligament reconstruc- of 134 children treated surgically showed that
tion [33], intrinsic muscle slide [34], or a resection preoperatively 50% had impaired two-point discri-
of the ulnar nerve motor branch in Guyon’s canal mination and 75% had impaired stereognosis;
[35]. The author’s preferred method is the lateral impaired sensibility had no adverse effect on
band rerouting procedure shown in Fig. 4, surgical results. Eliasson et al [18] reported on 32
because it requires less extensive dissection and children treated surgically with tendon transfers
rebalances the intrinsic and extrinsic tendon and muscle releases. Impaired sensibility before
deforming forces. the surgery did not influence the outcome. In fact,
Dahlin et al [38] reported 36 patients treated
operatively and followed for 18 months, finding
an improvement in stereognosis function associ-
Summary
ated with the improvement in their motor
The surgical results of upper extremity in- function, presumably because of improved func-
tervention have been shown to improve hand tional use.
function from paperweight/passive assist function Children with cerebral palsy can improve their
to active assist function [20]. Although children motor function and perhaps also their sensibility
with cerebral palsy commonly have a sensibility function with appropriately planned and executed
664 A.E. Van Heest / Hand Clin 19 (2003) 657–665

Fig. 4. (A) The extensor mechanism is incised longitudinally in the interval between the central slip and the lateral band
to allow the volar mobilization of the lateral band to the level of the volar plate. The A3 pulley is opened and sutured
closely around the lateral band, anchoring the lateral band volar to the axis of the PIP joint. The rerouting is tensioned
so that the PIP has light resistance to extension past 30 of flexion and does not extend past 5 of flexion. (B) Following
lateral band rerouting, this patient can actively extend his fingers without locking and can grasp his walker effectively.

tendon release and transfer surgery. Balance of wrist in spastic paralysis. A study of eighteen cases.
the wrist and fingers is the key element in im- J Bone Joint Surg 1966;48A(5):847–57.
provement of upper limb function. [4] White WF. Flexor muscle slide in the spastic hand:
the Max Page operation. J Bone Joint Surg 1972;
54B(3):453–9.
References [5] Green WT. Tendon transplantation of the flexor
carpi ulnaris for pronation-flexion deformity of the
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surgery. 2nd edition. Philadelphia: JB Lippincott; [6] Hoffer MM, Lehman M, Mitani M. Long-term
1979. follow-up on tendon transfers to the extensors of
[2] Van Heest A. Applications of botulinum toxin in the wrist and fingers in patients with cerebral palsy.
orthopedics and upper extremity surgery. Tech J Hand Surg 1986;11A(6):836–40.
Hand Upper Extrem Surg 1997;1(1):27–34. [7] Thometz JG, Tachdjian M. Long-term follow-up of
[3] Inglis AE, Cooper W. Release of the flexor-pro- the flexor carpi ulnaris transfer in spastic hemiplegic
nator origin for flexion deformities of the hand and children. J Pediatr Orthop 1988;8(4):407–12.
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[comment]. Dev Med Child Neurol 1998;40(9): Surg Clin N Am 1981;61(2):395–406.
612–21. [35] Manske PR. Cerebral palsy of the upper extremity.
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[22] Louis DS, Hankin FM, Bowers WH. Capitate- Surgery of the spastic hand in cerebral palsy.
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Hand Clin 19 (2003) 667–677

Surgical management of the thumb in cerebral palsy


Richard D. Lawson, MB, BS, FRACSa,1,
Michael A. Tonkin, MD, FRACSa,b,*
a
Department of Hand and Peripheral Nerve Surgery, Royal North Shore Hospital,
St. Leonards, NSW 2065, Australia
b
University of Sydney, Department of Surgery, Blackburn Building D06, St. Leonards,
NSW 2006, Australia

Deformity develops in cerebral palsy (CP) as may undergo fibrosis and develop a fixed de-
a result of muscle imbalance. The posture of the formity; this is termed a myostatic contracture.
thumb is determined by the balance of spasticity The skeleton fails to develop fully and the affected
and weakness in the intrinsic and extrinsic muscles thumb is typically 1.4–1.7 cm shorter than the
of the thumb, and the stability of the carpo- contralateral normal thumb [4].
metacarpal, metacarpophalangeal (MCP), and Keats stated that only 5% of the patients he
interphalangeal (IP) joints. The typical thumb examined would be helped by surgery [1].
deformities in CP are adduction contracture and Swanson later estimated that approximately
adduction–flexion contracture (thumb-in-palm 10%–20% of patients with upper limb involve-
deformity). ment in CP might benefit from surgery [3]. Careful
Thumb-in-palm deformity impairs grip and selection is necessary to identify this group.
appearance and in severe cases may affect hygiene.
Not only does the position of the thumb make it
difficult to place objects in the palm, it may excite Assessment
continued flexion of the fingers, compounding The examination of a child with CP is difficult
the usual finger flexion deformity [1]. The lack and requires patience and repeated assessments.
of thumb extension limits hand span and hence Because stress or excitement may increase spas-
the size of objects that can be grasped [2]. The ap- ticity, the surgeon must win the child’s trust by
pearance of the thumb is of importance to many providing a nonthreatening environment and
patients, with approximately 50% of patients enlisting the aid of other professionals. A skilled
seeking cosmetic improvement [3]. occupational therapist may be able to demon-
Natural history strate actions and abilities that had been masked.
Videotaping of the child engaged in play activities
Infants with thumb-in-palm deformity may is useful in assessing the child’s abilities. The
have a spontaneous resolution during the first parents’ input is vital.
year. If the deformity persists after the age of 1 The specific areas that need to be considered
year it is unlikely to resolve. The initial deformity include:
is dynamic, but with time the affected muscles
1. Etiology and pattern of CP
1 2. Manifestations in upper limb
Present address: Discipline of Pediatrics and Child
Health, University of Sydney, New South Wales, NSW
3. Specific patterns of involvement in thumb
2145, Australia. 4. Voluntary movement
* Corresponding author. 5. Sensory deficit
E-mail address: mtonkin@surgery.usyd.edu.au 6. Intelligence
(M.A. Tonkin). 7. Parental comprehension and expectations
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00042-8
668 R.D. Lawson, M.A. Tonkin / Hand Clin 19 (2003) 667–677

The diagnosis of CP should be confirmed, If the examiner is unsure about the function of
reassuring the clinician that the neurologic deficit the extensor and abductor muscles after a period
is static. of observation, a median nerve block at the elbow
The pattern of CP must be established. CP of may unmask extensor function [8].
the spastic variety can be treated with good The motion and stability of the thumb joints
results, but the athetoid variety should be can be assessed by documenting the passive range
approached with great caution. Although release of motion, the position of each joint during active
of contractures and stabilization of joints may be fist formation and active radial abduction with
indicated for reasons of hygiene [5], tendon simultaneous finger extension.
transfers have unpredictable results in athetoid House [5] and other authorities place great
CP and should be avoided. emphasis on the need to establish the muscle
The thumb-in-palm deformity usually occurs groups under voluntary control, considering this
in conjunction with other upper limb deformities, the most important determinant of success. The
and the treatment of the thumb should be part of child should be watched while performing a vari-
a coordinated strategy to improve the function ety of tasks. The ability to open the hand can be
of the limb. A plan must be developed to address assessed formally by measuring with disks of
the forearm pronation, wrist flexion, and finger various sizes from 6–18 cm, and the ability to
flexion deformities that often accompany thumb- grasp can be documented by observing the child
in-palm. Shoulder and elbow dysfunction also grasp balls of varying sizes. The varying types of
may need consideration. pinch should be assessed also [9].
To formulate a logical treatment plan it is Sensory deficit is common, particularly pro-
necessary to establish the muscles involved in the prioception, stereognosis, and vibration. Most
deformity and the motion and stability of the believe function and surgical results are superior
thumb joints. Assessment of individual muscle if good sensory function is present. Normal vision
function usually is achieved through observation may compensate for sensory deficits, however [5].
and palpation of the contracting muscle bellies, Rayan [10] found that sensory deficiencies did not
but electrophysiologic testing and selective nerve affect the results of his surgery.
blocks may have a role. It is also necessary to Some investigators believe that an intelligence
establish which muscles are available as motors if quotient of greater than 70 is desirable if surgery
tendon transfers are contemplated. In thumb-in- is contemplated [9]. Manske [11] and others, how-
palm deformity, the potential motors may include ever, have treated the deformity successfully with-
brachioradialis (BR), flexor carpi radialis (FCR), out reference to the child’s intellectual capacity.
flexor carpi ulnaris, palmaris longus (PL), and the The surgeon should ensure that the child and
superficial finger flexors [5]. Wrist flexors often are the parents have an understanding of the likely
unavailable, however, following rebalancing of effects of treatment and that unrealistic goals are
wrist and finger deformities. not set. The child will not gain fine, coordinated
The adductor pollicis (AP) is involved in most activity following surgery. [8]. Normal hand
cases of thumb deformity. The flexor pollicis function is not possible.
brevis (FPB) muscle is not always spastic and the
Classification
position of the thumb differs with involvement of
FPB. If involved, the resultant MCP joint flexion House published a classification of thumb-in-
causes the thumb to lie across the palm with its tip palm deformity in 1981 [5].
close to the base of the little finger; if not, the AP
causes the first web space to be tightly closed with Type I—simple metacarpal adduction contracture
the thumb and second metacarpal closely approx- Spasticity of the AP and first dorsal inteross-
imated [6]. The abductor pollicis brevis usually is eous causes adduction of the thumb metacarpal
not involved in spastic cerebral palsy but may be and first web space contracture. The MCP and IP
affected in athetoid CP [7]. joints are mobile.
The flexor pollicis longus (FPL) muscle is Type II—metacarpal adduction contracture and
involved variably. The thumb extensors typically metacarpophalangeal flexion deformity
are weakened and the tendons may be stretched. In addition to the contractures mentioned
The long thumb abductor (APL) is affected previously, FPB spasticity leads to flexion de-
similarly. formity at the MCP joint. The IP joint is mobile.
R.D. Lawson, M.A. Tonkin / Hand Clin 19 (2003) 667–677 669

Type III—metacarpal adduction contracture


combined with a metacarpophalangeal
hyperextension deformity or instability
The long and short thumb extensors act
across a mobile MCP joint to compensate for
the adduction deformity of the metacarpal, but
the result is hyperextension of the MCP joint. The
FPL is not spastic.
Type IV—metacarpal adduction contracture
combined with metacarpophalangeal and
interphalangeal flexion deformities
This deformity may arise from FPL spasticity
alone or in concert with spasticity of the thumb
intrinsics.
Type I deformity was the most common in
House’s series, and type IV deformity was most
severe
The senior author has proposed a modification
of this classification [12]. The patient is instructed to
make a fist while attempting to maintain the thumb
in a lateral pinch position. Observation then
permits assignment of the patient into one of three
groups:
Type 1—intrinsic deformity
The spastic intrinsic muscles (AP, FPB, and
the first dorsal interosseous) cause adduction of Fig. 1. Type 1: intrinsic deformity. (From Tonkin MA,
the thumb metacarpal, flexion of the MCP joint, et al. Surgery for cerebral palsy. Part 3: classification and
and extension of the IP joint. The APL and short operative procedures for thumb deformity. J Hand Surg
and long thumb extensors are paretic (Fig. 1). 2001;26B(5):465–70; with permission.)

Type 2—extrinsic deformity


The dominant deforming force is the FPL,
opposing a weak extensor pollicis longus (EPL). Surgical treatment
Metacarpal adduction is less marked. This pattern
of deformity is uncommon (Fig. 2). Principles of treatment
The key concept in the surgical treatment of
Type 3—combined deformity thumb-in-palm deformity is to rebalance the forces
There is spasticity of the intrinsic muscles and acting across the thumb joints by decreasing de-
the flexor pollicis longus, with weakness of the op- forming forces and augmenting opposing muscles.
posing muscles. The result is adduction of the Unstable joints may need stabilization or arthrod-
thumb metacarpal and flexion of the thumb MCP esis. All elements of the thumb-in-palm deformity
and IP joints, with a thumb-in-palm posture (Fig. 3). should be addressed in the same sitting, as serial
interventions have a higher risk for failure or
Nonoperative treatment
recurrence of the deformity [8].
Nonsurgical treatment may achieve limited Neurectomy (for example, of the deep motor
results. A rigid orthosis may maintain the thumb branch of the ulnar nerve) is not recommended;
out of the palm but at the expense of thumb it is preferable to weaken a muscle rather than
mobility. Softer splints can be used in mild defunction it totally [3], because this decreases the
deformities and can assist in maintaining thumb possibility of overcorrection. Release of a spastic
abduction while permitting some movement [12]. muscle dampens the overactive stretch reflex
Injection of botulinum toxin into the adductor and decreases spasticity, while preserving some
may aid in assessment and management. function.
670 R.D. Lawson, M.A. Tonkin / Hand Clin 19 (2003) 667–677

a deformity dating from infancy usually had


accepted the deformity and were not good surgical
candidates. Others believe the deformity can be
corrected at any age [8]. Manske [11] noted, in
a series of patients ranging from ages 5–17 years,
that the age of the patient at the time of surgery
was not related to the result.
It is usually appropriate to start proximally in
the plegic upper limb and work distally [12].
Shoulder and elbow function must be adequate to
position the hand in space [5]. If surgery to the
shoulder or elbow joints is proposed, this should
be performed before surgery on the thumb.
Although Zancolli [7] and other authorities
believe that the elbow, forearm, and hand should
be addressed in the same sitting, it is difficult to
position the limb correctly postoperatively if the
thumb and hand are treated concurrently; fur-
thermore, the position of the wrist and its motors
influence the thumb position and any proposed
transfers. The senior author therefore prefers to
operate on the thumb 6–12 months after correc-
tion of wrist deformity [12].
House addressed the thumb-in-palm deformity
using three steps [5]:

Step I—Release of contractures


Fig. 2. Type 2: extrinsic deformity. (From Tonkin MA,
Step II—Augmentation of weak muscles
et al. Surgery for cerebral palsy. Part 3: classification and
Step III—Joint stabilization
operative procedures for thumb deformity. J Hand Surg
2001;26B(5):465–70; with permission.)
Step I—release of contractures
Matev [13] considered release of the thenar mus-
cles (intrinsic release) to be the most important
The opposing muscles may be assisted by aspect of surgical treatment of thumb-in-palm
a combination of tenodeses, tendon rerouting deformity. Extrinsic flexor (FPL) involvement
and tendon transfers. Although not ideal for may require extrinsic release. The skin and dor-
tendon transfers, spastic muscles can be trans- sal fascia also may be contracted, necessitating
ferred and can maintain adequate function [5]. a four-flap or z-plasty of the first web space and
Pulp pinch is rarely achievable if not present release of the dorsal fascia [5].
preoperatively [3]. The objective should be to
obtain lateral or key pinch grip between the
Intrinsic release
middle phalanx of the index finger and the thumb
during grasping activities and thumb abduction Release of the insertion of AP into the ulnar
and extension during release. sesamoid may unduly weaken thumb flexion at
Additional goals are to improve the patient’s the MCP joint and lead to a swan neck deformity
psychologic well being [9] and to improve the of the thumb. Matev advocated release of the
appearance of the hand. origin of AP (along with FPB and most of the
abductor pollicis brevis) by way of a curved
Timing and staging of treatment
incision in the line of the thenar crease, extending
The pattern of deformity is usually apparent distally from the transverse carpal ligament. The
by age 3 years. The optimal time for surgery may flexor tendons and neurovascular bundles are
be at approximately age 5 or 6 years, when the retracted ulnarly, and the transverse head of AP
child is old enough to comply with postoperative released from its origin along the length of the
therapy. Zancolli [9] believed that adults with third metacarpal, working from distal to proximal.
R.D. Lawson, M.A. Tonkin / Hand Clin 19 (2003) 667–677 671

Fig. 3. Type 3: combined deformity. (From Tonkin MA, et al. Surgery for cerebral palsy. Part 3: classification and
operative procedures for thumb deformity. J Hand Surg 2001;26B(5):465–70; with permission.)

The muscle is displaced distally and radially. The The first dorsal interosseous may be released at
deep branch of the ulnar nerve and deep palmar its insertion or origin. Release from the first
arch is seen in the depths of the wound and metacarpal origin is the preferred procedure, as
protected. The thenar branch of the median nerve release from the second metacarpal may lead to
is identified and preserved [13]. The oblique head intrinsic dysfunction of the index finger. Matev
of AP, taking origin from the bases of the second [13] describes releasing the distal portion of the
and third metacarpals and the capitate and muscle from the ulnar border of the first meta-
trapezoid, is released also. If a deforming force, carpal using the thenar based incision for the
FPB may be released by way of the same thenar adductor release.
crease incision, at its origin from the trapezium
and the transverse carpal ligament (Fig. 4). Extrinsic release
House [5] prefers an intramuscular release of
the AP tendon through an incision in the first web A spastic FPL tendon can be weakened by
to avoid excessive weakening of the adductor, but a tendon slide at the musculotendinous junction.
states that longstanding and severe contracture A small incision is made in the distal volar
may need release of the adductor origin as forearm and the tendon is released; the thumb
described by Matev. This incision provides access IP joint is hyperextended and the tendon should
to the first dorsal interosseous and can be be seen to slide 1 cm distally (Fig. 5). FPL
combined with a four-flap Z-plasty. contracture may be managed by an intratendinous
Hoffer [14] performed electromyography of the Z-lengthening, with 0.5 mm of lengthening for
AP in a group of patients with contractures of the each degree of correction desired [4].
first web space to determine if the adductor
muscle contraction was continuous or selective. Step II—augmentation of weak muscles
AP did not contract during grip release in patients The options for augmenting APL, EPL, and
with selective firing. In this group of patients, extensor pollicis brevis (EPB) are many and need
Hoffer performed only a partial release of the to be directed toward the patient’s specific
muscle insertion, retaining the insertion of the problems. The main surgical procedures are
oblique head of AP. By performing a limited tenodeses, tendon rerouting to alter the direction
release he was able to retain side pinch. He found of action, and tendon transfers. If the EPL or EPB
that patients with selective firing of AP were over tendon is strengthened, stability of the MCP joint
weakened by a complete adductor release from its is essential to avoid the development of a swan
origin. neck deformity.
672 R.D. Lawson, M.A. Tonkin / Hand Clin 19 (2003) 667–677

Reeducation of transferred muscles may be


more unpredictable and difficult in patients with
cerebral palsy than those with peripheral nerve
lesions because of the underlying central de-
ficiency [11].

Abductor pollicis longus


The APL acts to abduct the thumb metacarpal.
An adduction posture can be treated by transfer
of BR, PL, or a tendon of flexor digitorum
superficialis [8]. APL usually has multiple slips.
During surgery these are pulled on in turn to
determine the slip that best draws the thumb out
of the palm (Fig. 6).
House advocated translocation of the APL
tendon palmar-ward through release of the first
dorsal compartment and strengthening of its
action by an end-to-side transfer of PL [5].
An alternative procedure is to divide the APL
tendon proximally. The distal end is directed
volarly and attached end-to-end with a suitable
motor (eg, PL) and the proximal end is attached
end-to-side under greater tension to EPB to
augment its action [5]. The biomechanics of such
a procedure may compromise EPB muscle con-
traction, however, simply replacing it with in-
Fig. 4. Incision for release of adductor pollicis. (From adequate APL contractility.
Tonkin MA, et al. Surgery for cerebral palsy. Part 3: If there is no satisfactory muscle for transfer,
classification and operative procedures for thumb de- another option is to produce a dynamic abductor
formity. J Hand Surg 2001;26B(5):465–70; with permis- tenodesis effect. APL is divided, the distal end is
sion.) attached end-to-side to FCR and the proximal

Fig. 5. Intramuscular slide of flexor pollicis longus (From Tonkin MA, et al. Surgery for cerebral palsy. Part 3:
classification and operative procedures for thumb deformity. J Hand Surg 2001;26B(5):465–70; with permission.)
R.D. Lawson, M.A. Tonkin / Hand Clin 19 (2003) 667–677 673

Fig. 6. Intraoperative traction to determine which tendon best positions the thumb. (From Tonkin MA, et al. Surgery
for cerebral palsy. Part 3: classification and operative procedures for thumb deformity. J Hand Surg 2001;26B(5):465–70;
with permission.)

end is attached to EPB [5]. APL also can be


tenodesed around brachioradialis or the first
dorsal compartment [2], tenodesed to the distal
radius [9], or plicated to increase the tension in the
muscle.

Extensor pollicis brevis


If the MCP joint is not hyperextensible after
release of any contractures, then plication of the
EPB tendon may be helpful in drawing the prox-
imal phalanx away from the palm [4]. Transfer
to EPB may be considered if the tendon is of
satisfactory size and intraoperative traction re-
sults in improved thumb position. The EPB also
can be tenodesed to FCR [5].

Extensor pollicis longus


The normal passage of EPL around Lister’s
tubercle imparts an adduction moment. By
rerouting the tendon more radially this moment
can be reversed.
Manske [11] detailed treatment of a group of
patients with thumb-in-palm deformity with
voluntary action of EPL and no fixed flexion
Fig. 7. The Manske procedure (A) Release and mobi-
deformity of the IP joint. He divided EPL 10 mm
lization of EPL. (B) Rerouting of EPL through the first
distal to the MCP joint and rerouted the tendon dorsal compartment. (C) Reattachment of EPL through
radial to Lister’s tubercle, through the first dorsal dorsal MP joint capsule to the extensor mechanism.
extensor compartment (or around the tendons of (Adapted from Manske PR. Redirection of extensor
EPB and APL if the compartment was too tight pollicis longus in the treatment of spastic thumb-in-palm
to accept passage of the EPL tendon) and then deformity. J Hand Surg [Am] 1985;10A(4):553–60).
674 R.D. Lawson, M.A. Tonkin / Hand Clin 19 (2003) 667–677

through a tunnel in the MCP joint capsule. The roughened surface of the palmar plate as it is
end of the tendon was then advanced 1 cm and advanced. The plate is fixed by way of pull-out
sutured under some tension back into the defect sutures tied dorsally, and the joint is stabilized in
in the extensor apparatus, with the thumb held 10 –15 of flexion with a temporary Kirschner
in abduction and extension (Fig. 7). wire [3,15].
The EPL tendon may be divided proximal to Alternatively, the sesamoid may be advanced
Lister’s tubercle and dissected free distally, then with the palmar plate. In Zancolli’s technique
passed through the first dorsal compartment and of sesamoid arthrodesis, the MCP joint is ap-
the ends rejoined under tension, with the thumb in proached using a curved dorsoradial incision
extension and abduction to produce a result and the palmar plate is mobilized after division
analogous to Manske’s operation [10]. of the accessory collateral ligament insertion. The
EPL may be reinforced by the transfer of BR or sesamoid is denuded of cartilage and fixed in a
a tendon of flexor digitorum superficialis [8], or PL. defect created at the head–neck junction of the
A superficialis tendon can be detached at the wrist metacarpal with the thumb in 30 of flexion. Two
and passed through the interosseous membrane to intraosseous sutures are passed across the sesa-
insert into EPL, or passed radially and subcutane- moid and tied over the dorsal surface of the
ously if EPL has been rerouted. Goldner felt that metacarpal. The joint then is stabilized with
FCR was too strong to use as a motor for EPL [8], a Kirschner wire that is removed at 5 weeks
but Swanson states that it is used commonly [3]. (Fig. 8A, B). In a series of 20 patients with MCP
joint hyperextension seen at an average of 32
Step III—joint stabilizations months after surgery, the preoperative hyperex-
The early procedures for thumb-in-palm de- tension averaged 38 and the postoperative
formity relied on arthrodeses of the carpometa- hyperextension 3 , whereas postoperatively 45
carpal and MCP joints, but the improvement in of flexion were retained [16] (Fig. 8C).
position was at the expense of thumb motion and Fusion is useful in recalcitrant thumb flexion
function [8]. Stabilization of the thumb joints now and hyperextension deformities [4]. The joint is
is performed only if they remain unbalanced after approached by way of a dorsoulnar incision and the
tendon release and transfers [5]. The MCP joint extensor apparatus incised. The joint is entered
most often needs attention. after detachment of the ulnar collateral ligament
from the metacarpal head. The articular cartilage is
Carpometacarpal joint removed with a scalpel and the epiphysis of the
proximal phalanx is preserved. The MCP joint is
Intermetacarpal bone block once was used to
fused in a position of 10 of flexion, 10 of
stabilize this joint, but this greatly limited thumb
abduction, and slight pronation [3]. The arthrod-
movement and is now performed not. If meta-
esis is stabilized by smooth crossed K-wires. If the
carpal adduction cannot be controlled by other
epiphyseal plate is not damaged, there is little
methods, a carpometacarpal arthrodesis is pre-
additional shortening of the thumb [4]. Before
ferred. This preserves some movement at the
fusion of a thumb joint, provisional fixation with
scaphotrapezial joint.
temporary K-wires can be useful to assess the
consequences of an irreversible procedure [12].
Metacarpophalangeal joint
In the presence of thumb MCP joint hyperex- Interphalangeal joint
tension, tendon transfers to augment thumb
The interphalangeal joint generally does not
extension lead to further metacarpal adduction
need stabilization, but this may be required in the
and MCP joint extension. Hyperextension of
event of a FPL rupture post-lengthening [5] or in
more than 20 warrants treatment [7]. Excess
the presence of a severe and intractable IP joint
extension of the joint may be addressed by
flexion contracture. The technique for fusion of
capsulorrhaphy, sesamoid arthrodesis, or fusion
the MCP joint is used.
of the MCP joint. The first two procedures involve
proximal advancement of the palmar plate and
Authors’ recommended procedures
maintain some thumb flexion.
In capsulorrhaphy the proximal attachment Type 1 (intrinsic) deformity
of the palmar plate is divided and a notch created Adductor/FPB release in the palm (younger
in the neck of the metacarpal to receive the patients) or by way of a dorsal approach
R.D. Lawson, M.A. Tonkin / Hand Clin 19 (2003) 667–677 675

Fig. 8. Sesamoid arthrodesis. (A) The palmar plate is mobilized by dividing the accessory collateral ligament at its
insertion into the palmar plate. The articular surface of the sesamoid is denuded of cartilage. A cortical defect is made at
the head–neck junction of the metacarpal. (B) Two straight needles loaded into a Kirschner wire driver are used to pass
a Prolene suture through the sesamoid-palmar plate complex and metacarpal neck to secure the sesamoid into the
cortical defect created. (C) The intraosseous suture is tied over the metacarpal under the extensor tendons as a permanent
suture. A Kirschner wire is placed across the MPJ to maintain the joint in approximately 30 flexion. The seam in the
collateral ligament is repaired and the proximal radial edge of the palmar plate is sutured to the metacarpal periosteum
and aponeurotic fibers of the abductor pollicis brevis. (From Tonkin MA, et al. Sesamoid arthrodesis for hyperextension
of the thumb metacarpophalangeal joint. J Hand Surg 1995;20A:334–8; with permission.)
676 R.D. Lawson, M.A. Tonkin / Hand Clin 19 (2003) 667–677

(older patients with established myostatic In Matev’s series, 3 of 21 patients had un-
contractures) satisfactory results, 2 of whom had athetosis. Of
First dorsal interosseous release after adductor the remainder, 6 patients obtained good grip and
release if required pinch and 12 had a thumb that did not interfere
Augmentation of thumb extension and abduc- with finger flexion and participated to some
tion with EPL reroute to EPB in combina- degree in grasp [13].
tion with BR-to-APL transfer In Tonkin’s 2001 series of 32 patients, the
Stabilization of MCP joint by way of capsu- procedures recommended previously were used.
lodesis if required; MCP joint fusion as The thumb was maintained out of the palm in 29
a salvage procedure patients and lateral pinch was obtained in 26
patients. None was made worse by the surgery [2].
Type 2 deformity
Improved position and function of the thumb
FPL tendon slide
may lead to improved stereognosis, perhaps
Other contractures are addressed as required
because of cortical reorganization following mod-
Type 3 deformity ified afferent input [17].
Adductor release in palm
First dorsal interosseous release if required Summary
FPL tendon slide
Augmentation of thumb extension and abduc- Surgical treatment of the thumb in cerebral
tion with EPL reroute to EPB in combina- palsy is complex and treatment must be directed
tion with BR-to-APL transfer toward the specific deformities. Thumb-in-palm
Stabilization of MCP joint by way of capsu- deformity can be treated successfully in patients
lodesis if required; MCP joint fusion as with spastic CP, but caution is required in patients
a salvage procedure with other varieties of CP. The main deforming
IP joint fusion if recalcitrant flexion deformity force is contracture and spasticity of the intrinsic
Postoperative care thumb muscles. Treatment involves release of the
deforming muscles and other soft tissue, augmen-
Matev [13] considered that after a properly tation of opposing muscles, and stabilization of
conducted operation, the thumb would be drawn appropriate joints.
out of the palm with the MCP and IP joints
slightly flexed. The thumb is maintained in full
radial abduction and 20 of palmar abduction for References
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[1] Keats S. Surgical treatment of the hand in cerebral
thumb, it is important to ensure that abduction of
palsy: correction of thumb-in-palm and other
the metacarpal is occurring, rather than hyperex-
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A removable splint is used thereafter while the [2] Tonkin MA, Hatrick NC, Eckersley JRT, Couzens
hand therapist works with the child to reeducate G. Surgery for cerebral palsy. Part 3: classification
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[3] Swanson AB. Surgery of the hand in cerebral palsy.
In: Flynn JE, editor. Hand surgery. Baltimore:
Results Williams and Wilkins; 1982. p. 476–88.
[4] Goldner JL, Koman LA, Gelberman R, Levin S,
Results are difficult to describe and interpret Goldner RD. Arthrodesis of the metacarpophalan-
because the pathology and preoperative hand geal joint of the thumb in children and adults.
function varies so widely. Furthermore, functional Adjunctive treatment of thumb in palm deformity
and cosmetic improvement results from finger, in cerebral palsy. Clin Orthop 1990;253:75–89.
[5] House JH, Gwathmey FW, Fidler MO. A dynamic
wrist, and more proximal limb surgery.
approach to the thumb in palm deformity in
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Churchill Livingstone; 1987. p. 153–68. contracture of the thumb in cerebral palsy. Acta
[8] Goldner JL. Upper extremity tendon transfers in Orthop Scand 1970;41:439–45.
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389–414. Adduction contracture of the thumb in cere-
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the spastic hand in cerebral palsy: report of the 755–9.
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[Am] 1983;8A(5 Part 2):766–72. metacarpophalangeal joint of the thumb in children
[10] Rayan GM, Saccone PG. Treatment of spastic with cerebral palsy. J Bone Joint Surg [Br] 1976;
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pollicis longus tendon rerouting. J Hand Surg [16] Tonkin MA, Beard AJ, Kemp SJ, Eakins DF.
[Am] 1996;21A(5):834–9. Sesamoid arthrodesis for hyperextension of the
[11] Manske PR. Redirection of extensor pollicis longus thumb metacarpal joint. J Hand Surg [Am] 1995;
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Hand Clin 19 (2003) 679–686

Functional and cosmetic outcome of surgery for


cerebral palsy in the upper limb
Bruce R. Johnstone, MBBS, FRACSa,*,
Philip W.F. Richardson, MBBS, FRACSa,
Christopher J. Coombs, MBBS, FRACSa,
Josie A. Duncan, BAppSc, OTb
a
Department of Plastic and Maxillofacial Surgery, Royal Children’s Hospital, Parkville, Victoria 3052, Australia
b
Department of Occupational Therapy, Royal Children’s Hospital, Parkville, Victoria 3052, Australia

Cerebral palsy is a group of nonhereditary or paralysis of deforming spastic muscles, tendon


neurologic disorders caused by prenatal, perina- transfers, and joint stabilizations [9]. The goals of
tal, or postnatal insults to the motor portion of surgery are to improve cosmesis [4,6,7,10–12],
the central nervous system [1]. It is characterized function [4,5,11,13], ease of dressing, and ability
by paralysis, weakness, loss of coordination, and to clean the limb [12], while minimizing surgery-
defective motor control, and also can include related scars. Completely normal hand function
sensory loss, neglect, developmental delay, seiz- cannot be achieved [14]. Disappointment with the
ures, and learning disabilities [2]. results of surgery usually is a result of unrealisti-
Most patients have spastic cerebral palsy and cally high expectations [7]. The highest levels of
show increased tone, reduced voluntary control, postoperative satisfaction can be achieved by
and hyperactive stretch reflexes; these patients preoperatively establishing appropriate expect-
tend to benefit most from surgery as compared ations in the minds of the caregivers and the
with those with movement disorders such as patient.
dystonia. Thorough assessment is critical [3,4] Planning surgical treatment is complex, as no
and this should define the patient with potential two patients are alike [3]. Many but not all children
for worthwhile improvement. This is best done in with cerebral palsy are surgical candidates [5,9].
a multidisciplinary setting that includes plastic Those children most likely to benefit are motivated
surgeons, orthopedic surgeons, hand therapists, [1,11], have sufficient IQ and emotional stability
and physiotherapists [5]. It is essential to establish [1,6,15], and do not have neglect [11]. Patients with
what the patient can and cannot do and what spastic cerebral palsy [7,15,16] and those with good
specific problems they face, including dressing and sensibility [1,7,15], voluntary control, and adequate
hygiene. Cognitive capacity, neglect, and sensation strength [7] tend to do better. Those with athetosis,
of the limb also are noted, as these affect outcome. dystonia, and neglect [2,7,16] may benefit from
Nonoperative management options include static procedures such as tenodeses, joint stabiliza-
casting, splinting, muscle stretching, muscle tions, and arthrodeses.
training, reeducation and rehabilitation [5–7], The results of nonoperative treatment have been
and botulinum toxin injections [8]. Principles of acceptable in selected patients with specific indica-
surgical management include release, lengthening, tions [8,17,18]. The results of surgery are variable,
and Rosenbaum in 1990 [19] noted an absence of
validated measures of outcome. A 0–8 scale of
* Corresponding author. 7th Floor, 766 Elizabeth functional level was developed by House in 1981 [3].
Street, Melbourne, Victoria 3000, Australia. Van Heest et al [11] reported an improvement in
E-mail address: bjkr@bigpond.com (B.R. Johnstone). function of 2.6 levels on average using this scale in
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00031-3
680 B.R. Johnstone et al / Hand Clin 19 (2003) 679–686

134 patients over 25 years, as assessed by clinicians. The authors’ study consisted of two parts. First,
They stressed the use of multiple simultaneous a retrospective review was conducted of all 68
procedures averaging 4 per operation. Better out- patients operated on by the senior investigator
comes were seen when original function was fair to between April 1992 and April 2001 for upper limb
good. Sensibility did not seem to affect outcome. spasticity. Fifty-one patients had spastic hemi-
House et al in 1981 [3] reported that the age of plegic cerebral palsy, 12 had spastic quadriplegic
surgery made little difference in outcome. Roth et al cerebral palsy, and 5 had either strokes or head
[20] showed on average a functional improvement injuries leading to spastic hemiplegia. A total of
of 1.8 on the modified House Scale [9], again, as 52% of patients were female. Surgery was per-
judged by clinicians. They also looked at parental formed on the right upper limb on 41 occasions, on
assessment and 65% believed there was definite the left on 33 occasions, and on both on 5 occasions.
improvement and 35% some improvement. Fifteen Data were collected on types and numbers of
of 17 parents were satisfied, but this was not related procedures per operation, age at surgery, anesthetic
to functional levels after surgery or degree of duration, and date of operation.
improvement. Mowery et al in 1985 [22] studied Second, a telephone interview was conducted
12 patients and noted improved appearance of the with the parents, caregivers, or patient, depending
hand according to ‘‘several parents’’ and that on who was most appropriate. Outcomes of their
adolescents no longer kept their hand hidden. surgery were scored in function, cosmesis, ease of
Improved self care, grasp, and cosmesis were noted dressing, and hygiene, relative to their preopera-
by Eliasson et al in 1998 [10], who reviewed 32 tive status. Overall satisfaction also was assessed.
children, and Dahlin et al 1998 [20] reported ex- These questionnaires were administered by a re-
pectations fulfilled in 28 of 29 parents followed for search fellow not involved with their surgery. The
18 months. Tonkin et al 1992 [4] reported that 30 of validated House scale [3,9,20] was used for
34 patients had an improvement in function or functional assessment (Table 1). New scales were
cosmesis but these did not necessarily go together. developed because of the paucity of validated
Samilson et al in 1964 [11] showed improved scales for cosmesis, dressing, and hygiene (Tables
function and cosmesis in 40 patients, whereas 2–4). Descriptions were selected based on the
Keenan et al in 1987 [12] showed improved func- language used by many of the patients and
tion, cosmesis, and hygiene in 27 patients. These caregivers to describe their problems before and
results contrast with those of Phelps who, in 1957 after surgery.
[23], after operating on 242 patients, concluded
that tendon transfers usually failed.
There is, however, a paucity of knowledge on
Surgery
the effects of surgery on patients’ function,
cosmesis, hygiene, and ability to dress, from A comprehensive preoperative surgical plan is
a patient/caregiver perspective. Information about important and must aim to rebalance the upper
caregiver-based outcomes in these areas conveys limb at all levels. This plan is based on a thorough
what can be realistically achieved and provides preoperative assessment by a hand surgeon and
realistic individual goals. Furthermore, there is hand therapist. A video of the patient allows
little in the literature regarding patient/caregiver documentation of the preoperative status and
overall satisfaction. fine-tuning of the surgical plan.

Table 1
Function
Score Category Description
0 Does not use Does not use
1 Poor passive assist Uses as stabilizing weight only
2 Fair passive assist Can hold object held in hand but not grasp
3 Good passive assist Can hold object and stabilize it for use by other hand
4 Poor active assist weakly Can actively grasp object and hold it
5 Fair active assist Can actively grasp object and stabilize it well
6 Good active assist Can actively grasp object and manipulate it
7 Spontaneous use, partial Can perform bimanual activities and occasionally uses the hand spontaneously
8 Spontaneous use, complete Uses hand completely independently without reference to the other hand
B.R. Johnstone et al / Hand Clin 19 (2003) 679–686 681

Table 2
Dressing
Score Category Description
0 Very difficult Completely dependent on caregivers; requires extensive modification to
clothing, tight muscles causing distress getting arms through
1 Difficult Can wear normal clothes but dependent on caregivers; some difficulty getting
arms through but no distress
2 Moderate assistance Completely dependent but not difficult
3 Minimal assistance Child does most of the dressing
4 Completely independent No help required if clothes are selected for them

More recently, the Melbourne assessment of plied for elbow releases and more distal proce-
unilateral function in the upper limb in cerebral dures. These are completed in a swift and systematic
palsy [24] has been used for planning and to fashion. Meticulous skin closure with subcuticular
document function objectively as part of a pro- absorbable suture is completed to minimize post-
spective study. operative scarring. An epidural catheter is placed
It is important to prevent fixed deformities of in the brachial plexus for local anesthetic infusion
the shoulder, elbow, forearm, and wrist, and the following any axillary surgery. Accurate and safe
authors do this with passive ranging, serial splints immobilization is achieved with a padded plaster
and casts, and botulinum toxin. Obtaining a bal- of Paris splint in the appropriate position. A
anced wrist, preferably with active extension, is an plaster slab optimally immobilizing the thumb,
essential foundation for improving thumb and fingers, and wrist is allowed to set before an
finger posture. The variety of procedures used on above-elbow ‘‘sugar tong’’ cast is applied to hold
the upper limb reflects the complex and unique the forearm supinated with the elbow flexed to
patient profiles. The preoperative plan sometimes 90 . Patients who have had elbow flexor length-
may include botulinum toxin injections or phenol ening and pronator releases are immobilized in
injections of motor nerves in combination with a compromised position of 45 of elbow flexion. A
surgery. body spica is not required for shoulder proce-
The treatment of fixed deformities is challeng- dures, as caregivers are instructed in frequent
ing and must address skin, musculotendinous and passive shoulder abduction and external rotation.
aponeurotic structures, joint capsule, and bone. Postoperatively, the plaster is maintained for 4
The humerus or forearm bones may need osteot- weeks and then replaced by a thermoplastic splint
omies, whereas the wrist may require a proximal that is removed initially only for daily baths but
row carpectomy. Tendon transfers to augment progressively for longer periods until it is used as
wrist extension and radial deviation may be a night splint only in the third postoperative
limited in effect because of abnormal axes of month. Early scar hypertrophy is treated with
wrist motion. Rarely would the authors recom- silicon gel pads and pressure.
mend wrist fusion.
Results
Operative technique
Surgical data
Access incisions are planned for all procedures
to minimize length but maximize the maneuvers The surgical details on 84 limbs in 68 patients
performed through them. Shoulder procedures are were analyzed. The average age at the time of
performed before a pneumatic tourniquet is ap- surgery was 15.6 years and ranged from 6–47

Table 3
Hygiene
Score Category Description
0 Impossible to clean Can’t get into armpit/hand, unable to cut nails
1 Severely difficult to clean Smelly, skin breakdown problems, extensive skin buildup
2 Moderately difficult Occasionally smells, occasional skin breakdown and debris buildup
3 Minimal difficulty Rarely smells, no skin problems, rare debris buildup
4 No hygiene problems No hygiene problems
682 B.R. Johnstone et al / Hand Clin 19 (2003) 679–686

Table 4
Cosmesis
Score Category Description
0 Severe deformity Contractures at rest, extremely embarrassing for patient/caregiver, very low self-esteem
1 Moderate deformity Contractures obvious, embarrassing, low self-esteem
2 Mild deformity Contractures present, mildly embarrassing
3 Minimal deformity No deformity at rest, if using hand, one can tell there’s a problem
4 No deformity No deformity at any time

years. Follow-up duration ranged from 6 months cosmesis (P < 0.001) (Fig. 1). Function improved
to 8 years, 10 months, with an average of 3 years, from a median of 2 to 4 using the 0–8 House scale.
11 months. The mean number of operations Hygiene increased from a median of 2 to 3 on the
required per patient was 1.2. Ten patients had 0–4 scale. The median score for ability to dress did
more than one operation and no patients required not change, but the mean increased from 2.54 to
more than three operations. The average number 3.08. Cosmesis was most dramatically improved
of procedures per operation was 4.5 and ranged from a median of 1 to 3 on the 0–4 scale.
from 1–12, all of which were completed in a single Using the Two-sample Wilcoxon rank-sum
tourniquet run. Each operation had an anesthetic (Mann-Whitney) test, the patients were subdi-
time of 78 minutes on average, with a range of 10– vided first into two groups according to age more
240 minutes. The most common procedures were than or less than 14 years. There was no statistical
the pronator teres release and Green transfer. A difference in change in function (P = 0.88),
total of 374 procedures have been performed, with dressing (P = 0.0517), hygiene (P = 0.12), cos-
22 involving the shoulder, 39 the elbow, 74 for mesis (P = 0.90), or level of satisfaction (P = 0.24)
pronation, 93 to rebalance the wrist, 17 for the between the two groups.
finger flexors, 9 for the extensors, and 120 to With respect to patients with hemiplegia versus
correct the thumb (Appendix). other types of cerebral palsy, improvement in
cosmesis was significantly more in the hemiplegic
Questionnaire results group with a median change of 2.0 compared with
1.0 (P = 0.0014). There was no significant differ-
Forty-eight patients or their caregivers (71%)
ence between the two groups with respect to
could be contacted for a telephone interview re-
function (P = 0.58), hygiene (P = 0.38), dressing
garding outcome assessment. Forty-one (85.4%)
(P = 0.48), and satisfaction (P = 0.31).
believed that the outcome was worthwhile, with
With respect to time since the last operation
26 (54.2%) reporting a good or excellent result
(less than or greater than 3.5 years), there was no
(Table 5).
difference between the groups with respect to
Statistical analysis function (P = 0.090), hygiene (P = 0.17), cos-
mesis (P = 0.53), dressing (P = 0.98), and satisfac-
Results were analyzed using the nonparametric tion (P = 0.63).
sign test. There was a statistically significant Gender made no difference in outcome in
increase in functional status (P < 0.001), hygiene function (P = 0.66), hygiene (P = 0.20), cosmesis
(P < 0.001), ability to dress (P < 0.001), and (P = 0.71), dressing (P = 1.0), and satisfaction

Table 5
Overall satisfaction results in 48 patients
Score Category Description Number of patients
0 poor worse than preoperative 2 (4.2%)
1 marginal was not worth doing 5 (10.4%)
2 minor worthwhile gain little improvement, but worthwhile 15 (31.3%)
3 good results reached expectation 22 (45.8%)
4 excellent results better than expected 4 (8.3%)
B.R. Johnstone et al / Hand Clin 19 (2003) 679–686 683

Fig. 1. Graphs showing pre- versus postoperative scores of function, cosmesis, ability to dress, and ease of cleaning.
These box plots display the non-normality of the distribution of the variables. The change in the median and the range of
distribution are displayed. The median (50th percentile) is marked with a solid line. The boxes represent the 25th to 75th
percentile range and the T shaped ‘‘whiskers’’ extend to the closest data point within 1½ times the interquartile range.
The o indicates an extreme value. The median score for dressing did not change but the mean increased from 2.54 to 3.08.

(P = 0.51). It was not possible statistically to In surgical candidates, other investigators have
compare changes between patients with lower shown improvements in function [9,11,12,15] and
levels of function with those with higher levels cosmesis [11,12,21] as judged by health profes-
because of regression toward the mean. sionals.
Quantitative patient- or caregiver-based as-
sessment in the fives areas of function, dressing,
Discussion
hygiene, cosmesis, and overall satisfaction has not
Patients with cerebral palsy encounter problems been published previously. The authors’ assess-
with upper limb function and cosmesis, and some ment of function used the validated House scale
have problems getting dressed and cleaning the [9,17,22]. The authors’ scales for assessing other
axilla and hand [14]. Not all patients are surgical areas have been based on the terminology used by
candidates [15]. Complicating factors include re- caregivers or patients in clinic before the start of
duced sensation in approximately 50% of patients this study.
[25] hemineglect, reduced IQ [14], elements of The important findings from this study were as
extrapyramidal movement disorder [15], and fixed follows. First, the authors agree with Van Heest
deformity. and House [9] that a large number of procedures
684 B.R. Johnstone et al / Hand Clin 19 (2003) 679–686

are possible during one operation. This allows cerebral palsy. J Bone Joint Surg 1981;63A(2):
fewer anesthesias and hospital visits. Second, the 216–25.
authors have shown a significant improvement in [4] Tonkin M, Gschwind C. Surgery for cerebral palsy:
function, cosmesis, hygiene, and ease of dressing, part 2. Flexion deformity of the wrist and fingers.
J Hand Surg 1992;17B:396–400.
as assessed by caregivers after considerable
[5] Koman LA, Gelberman RH, Toby EB, Poehling
follow-up. Furthermore, the authors have shown GG. Cerebral palsy: management of the upper
high overall satisfaction levels, with 41 of 48 extremity. Clin Orthop 1990;253:62–89.
(85.4%) patients followed reporting that surgery [6] Swanson AB, Rapids G. Surgery of the hand in
was worthwhile. Hemiplegic patients tended to cerebral palsy and the swan neck deformity. J Bone
show larger improvements in cosmesis compared Joint Surg 1960;42A(6):951–64.
with quadriplegics. With time, patients remained [7] McCue FC, Honner R, Chapman WC. Transfer of
satisfied with the outcomes. the brachioradialis for hands deformed by cerebral
These results are clinically relevant. They allow palsy. J Bone Joint Surg 1970;52A(6):1171–80.
clinicians to counsel new patients and caregivers [8] Wall SA, Temlett LA, Perkins B, Becker P. Botuli-
num A chemodenervation: a new modality in cere-
about the potential long-term outcome in areas of
bral palsied hands. Br J Plast Surg 1993;46:703–6.
upper limb function, cosmesis, and ability to dress [9] Van Heest AE, House JH, Cariello C. Upper
and clean. Patients can be given more specific extremity surgical treatment of cerebral palsy.
goals of surgery, depending on their age and type J Hand Surg 1999;24A:323–30.
of limb involvement, what areas may be im- [10] Eliasson AC, Ekholm C, Carstedt T. Hand function
proved, and by how much. Caregivers and pa- in children with cerebral palsy after upper limb
tients can be reassured that most patients who tendon transfer and muscle release. Dev Med Child
have had upper limb surgery for spasticity have Neurol 1998;40:612–21.
been glad they had it done. [11] Samilson RA, Morris JM. Surgical improvement of
the cerebral palsied upper limb. J Bone Joint Surg
1964;46A(6):1203–16.
[12] Keenan MAE, Abrams RA, Garland DE, Waters
Summary
RL. Results of fractional lengthening of the finger
Function, cosmesis, hygiene, and ability to flexors in adults with upper limb spasticity. J Hand
dress can be improved by appropriate surgery. Surg 1987;12A:575–81.
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unbalanced wrist in cerebral palsy by tendon
procedures and addresses the muscles, their
transfer. Ann Plast Surg 1995;35:90–4.
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palsy: correction of thumb in palm and other
deformities. J Bone Joint Surg 1965;47A(2):
274–84.
Acknowledgments [16] Zancollli EA, Goldner LJ, Swanson AB. Surgery of
the spastic hand in cerebral palsy: Report of the
The authors thank Professor John Carlin, Committee On Spastic Hand Evaluation. J Hand
Director of the Clinical Epidemiology and Bio- Surg 1983;8(5 part 2):766–72.
statistics Unit of the Murdoch Children’s Re- [17] Koman LA, Williams RMM, Smith BJ, Kroening
search Institute, Melbourne, and Gabrielle Davie L, Shilt JS. Clinical and subjective outcomes
for their statistical analysis. following upper extremity botulinum toxin injec-
tions: preliminary experience and validation of
a physician administered outcome tool. Cong Int
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Appendix
Surgical procedures and numbers ECU stabilization 1
Dorsal capsule plication 1
Shoulder Radial styloidectomy 1
Pectoralis major release 7 ECRB tenodesis 1
Subscapularis release 6 ECRL tenodesis 1
Biceps short head release 4 ECRL to FCR transfer 1
Coracobrachialis release 4 ECRL to APL/EPL transfer 1
Pectoralis minor release 1 ECU to ECRL 1
FCU to ECRL transfer 1
Elbow
Biceps and brachialis lengthening 15 Finger flexors
Phenol musculocutaneous nerve 9 Lengthening 6
Brachioradialis release 8 STP (superficialis to profundus transfer) 6
Botulinum toxin (elbow flexors) 5 Botulinum toxin 3
Crush musculocutaneous nerve 1 Carpal tunnel release 1
Neurectomy musculocutaneous nerve 1 Median and ulnar neurectomy 1

Forearm Finger extensors


Pronator teres release 31 Side-to-side coaption 5
Bicipital aponeurosis release 19 FCU augmentation 2
Pronator teres reroute 16 BR augmentation 1
Interosseous membrane release 6 EDC tenolysis 1
Flexor origin release 1
Botulinum toxin pronator teres 1 Thumb
Adductor release 17
Wrist EPL reroute 13
Green transfer (FCU to ECRB) 48 APL tenodesis 11
FCR release 13 Sesamoid arthrodesis 10
Arthrodesis 7 PL/EPL augment 9
FCU release 4 FDI release 9
PL release 3 First web Z plasty 8
ECRL/B release/lengthen 2 FDS opponensplasty 7
Proximal row carpectomy 2 Manske EPL reroute 7
Volar capsule release 2 FPL lengthen 6
ECU release 1 Crush terminal ulnar nerve 6
BR to ECRL transfer 1 MPJ arthrodesis 5
Botulinum toxin wrist flexors 1 IPJ arthrodesis 2
686 B.R. Johnstone et al / Hand Clin 19 (2003) 679–686

EPL tenolysis 2 APB tenodesis 1


Botulinum toxin first web 2 Extensor tenolysis 1
APL to EPL transfer 1 EIP to EPL transfer 1
EPL plication 1
FDS to APL transfer 1 Total 374
Hand Clin 19 (2003) 687–699

Brachial plexus dorsal rhizotomy in


hemiplegic cerebral palsy
Jayme Augusto Bertelli, MD, PhDa,b,c,*,
Marcos Flávio Ghizoni, MDa,b,d, Thatiana Rodrigues Frasson, PTb,
Karla Samara Fernandes Borges, PTb
a
University of the South of Santa Catarina, Unisul, Department of Biological and Health Science,
CCBS, Praca Getulio Vargas, 322, Florianópolis, SC 88020030, Brazil
b
Joana de Gusma~o Children’s Hospital
c
Governador Celso Ramos Hospital, Department of Orthopedic Surgery, Praca Getulio Vargas,
322 Florianópolis, SC 88020030, Brazil
d
Nossa Senhora da Conceiça~o Hospital, Department of Neurosurgery, Praca Getulio Vargas,
322, Florianópolis, SC 88020030, Brazil

Historical aspects and evolution of posterior abolition of the tonic reflexes of the neck. In this
rhizotomy for spasticity relief connection, Heimburger et al [6] operated on 15
patients and added partial section of C4 to the
Posterior rhizotomy for relief of spasticity first
procedure. Spasticity of the neck, spine, arms, and
was reported experimentally by Sherrington in
legs decreased in 13 of the 15 patients. The
1898 [1] and later in 1908 when Foerster [2]
improvement was not dramatic in any of them,
introduced it into clinical practice. Foerster
however. Fraioli et al [7] increased the extent of the
credited Monro with performing similar work as
rhizotomy, sectioning bilaterally from C1 to C6.
early as 1904. At that time, Foerster proposed for
Their patients presented with athetosis and dysto-
the treatment of upper limb spasticity dorsal
nia. Despite the achievement of useful results, the
rhizotomy of C4 to T2 preserving C6, or al-
authors call attention to the high rate of re-
ternatively partial dorsal rhizotomy of all these
spiratory complications caused by the large num-
roots. Foerster stated that the effect of posterior
ber of sectioned dorsal roots. The results for
rhizotomy was likely to be better in the lower limb
cervical rhizotomy were not entirely satisfactory.
than in the upper limb [3]. In the last decade,
Benedetti et al [8] therefore combined upper
major interest in this topic has emerged and
cervical dorsal rhizotomy and partial rhizotomies
posterior rhizotomy now is frequently used to
of C4 to C7 in a two-stage surgery, reporting a good
treat lower limb spasticity [4]. The literature has
result in one patient.
less to say about the treatment of upper limb
In 1976, Fasano et al [9] described a new
spasticity by cervical dorsal rhizotomy.
technique of posterior rhizotomy for treatment of
In 1970, Kottke [5] reported on six patients
spasticity in the lower limbs. They stimulated fasci-
whose spasticity diminished after bilateral C1–C3
cles of the posterior roots electrically and found
posterior rhizotomy. He attributed the result to
that some fascicles responded to stimulation with
tonic muscle contractions. They cut these fascicles,
* Corresponding author. Governador Celso Ramos
preserving those with a weaker reaction or with no
Hospital, Department of Orthopedic Surgery, Praça reaction. Laitinen et al [10] used a similar technique
Getulio Vargas, 322 Florianópolis, SC, 88020030, on one patient with spasticity of the arm, sectioning
Brazil. 60%–80% of C6–C8 dorsal rootlets. The patient
E-mail address: bertelli@matrix.com.br (J.A. Bertelli). showed a good reduction of spasticity. In this
0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00038-6
688 J.A. Bertelli et al / Hand Clin 19 (2003) 687–699

connection, Xu et al [11] operated on 12 patients, fore the sole anatomic site at which muscle afferents
dividing 30%–60% of the posterior rootlets. These run separately from the motor efferents. Here, the
investigators claim a spasticity resolution of 95%. afferents can be divided surgically without section-
Alternatively, Sindou et al [12] proposed a selec- ing the efferents. Because regeneration of the dorsal
tive drezotomy for the treatment of spasticity. In root fiber into the spinal cord is abortive, there is no
1986, this author (ie, Sindou) reported good results need to remove any portion of the roots. Indeed,
from the procedure at the level of the brachial simple crushing is sufficient to promote a permanent
plexus in 16 hemiplegic patients [13]. Barbaro [14], lesion (for review, see [21]).
however, suggested that this procedure to the spinal
cord should be used in painful conditions rather
Anatomic basis of brachial plexus
than in spasticity, because rhizotomy alone is
dorsal rhizotomy
a more straightforward and less risky technique.
Based on modern reconstructive approaches to Upper limb innervation arises from the bra-
brachial plexus injuries and anatomic studies in chial plexus that is formed by the union of C4
animals, the authors recently have performed to T2 ventral and dorsal roots. Nonetheless,
brachial plexus dorsal rhizotomies for the treat- the main roots are C5 to C8. The proprioceptive
ment of upper limb dysfunction following CNS ganglionic neurones are situated at the same root
injury. Sixty-one patients have been operated on as the motor neurone pool in the spinal cord. The
with encouraging results [15,16]. exteroceptive ganglionic neurones present a more
even distribution along the roots. As a general
rule, a muscle is innervated by two roots, whereas
Pathophysiologic basis of brachial plexus dorsal
a dermatome receives contributions from three
rhizotomy
roots [22]. Dorsal root section at two levels
Damage to the CNS produces abnormal upper therefore abolishes muscle afferent information
limb function secondary to spasticity, paresis, but preserves dermatome sensibility [15,16].
general neurologic disorders, and disturbances C5, C6, and C7 emerge from the intervertebral
in hand sensibility. These abnormal signs evolve foramen above the vertebrae of their same
into a clinical syndrome known as upper motor number. C8 emerges from the intervertebral
neurone syndrome. The clinical features of move- foramen between C7 and T1. Hence, a three-level
ment dysfunction in upper motor neurone syn- hemilaminectomy from C5 to C7 allows exposure
drome are positive and negative. Negative features of the C5 to C8 dorsal roots. The dorsal roots are
correspond to loss of dexterity and weakness. Posi- formed by closely compacted filaments called
tive features consist of spasticity, which is related to rootlets. Between these rootlets, vessels are ob-
the tonic stretch reflex release and hyperreflexia and served easily, especially in the pediatric patient,
clonus, which are based on the phasic stretch reflex and should be preserved during surgery.
release. The release of cutaneous and nociceptive The brachial plexus is divided functionally into
reflexes, including the flexor withdrawal reflex and three groups: C5 and C6 innervating shoulder and
extensor reflexes, are also positive signs. Hyperex- elbow, C8 and T1 controlling hand function, and
citability of spinal reflex forms the basis of most of C7 functionally innervating shoulder, elbow, wrist,
the positive clinical signs, which have in common and hand. C7 traditionally has been related to the
excessive muscle activity [17]. All of these disin- radial nerve [23]. When C7 motor rootlets are
hibited spinal reflexes have an afferent pathway, sectioned for transfer in brachial plexus repair,
entering the spinal cord by way of the dorsal roots. however, the authors have observed in 80% of
The law of Bell and Magendie states that ventral patients a temporary decrease in grasping and
spinal roots contain only efferent or motor fibers pinch strength [24]. This means that C7 plays a role
and dorsal roots contain only afferent or sensory in extrinsic finger flexor muscle innervation. C7 is
fibers. The discovery of this separation of function also the root responsible for pronator teres and
occurred in the early 1800s and is one of the flexor carpi radialis innervation [25]. On that
principles of modern neuroanatomy [18]. Recently, account, when the shoulder and elbow are spastic,
afferents have been demonstrated in the ventral C5, C6, and C7 dorsal roots are divided. If the
roots [19]. The functional significance of these spasticity in the wrist and hand is more pro-
afferents is not yet clear, but evidence suggests that nounced, C8 dorsal rhizotomy should be added. If
these fibers mediate radicular and leptomeningeal shoulder adductors are not involved but elbow,
mechanosensitivity [20]. The dorsal root is there- wrist, and digital muscles are spastic, C6 to C8
J.A. Bertelli et al / Hand Clin 19 (2003) 687–699 689

dorsal rhizotomy is performed. Any involvement eyes open and closed, to detect any disturbance in
of finger flexors in the spastic disease calls for a C8 coordination. They then were asked to raise their
dorsal rhizotomy. When only the wrist and finger arm, subsequently flexing and extending the
flexors are spastic, C7 and C8 are divided. T1 is elbow, wrist, and fingers. Finally, they were asked
never divided, because adding a fifth section to the to pronate and supinate. Alternatively, they were
procedure might jeopardize hand sensibility. Spas- told to kiss the palmar and dorsal sides of their
ticity in hand intrinsic muscles, leading to intrinsic hand. During this phase, goniometry was per-
plus deformity is, in the authors’ experience, an formed. The patient was requested to walk and
exceptional situation. The authors have found it the degree of elbow flexion was measured.
only in poststroke patients and at no time in Patients were videotaped and the goniometry
patients with cerebral palsy. In this situation, was checked further in digital images.
however, a T1 dorsal rhizotomy should be con-
Spasticity and athetosis evaluation
sidered but C8 should be spared. In cerebral palsy,
when the thumb intrinsic muscles (C8 and T1 Patients were assessed sitting on a chair or,
innervation) are involved, being a component of depending on their age, sitting on their mothers’
the classic thumb-in-palm deformity, orthopedic knees. Muscle stretching was tested with variable
procedures are preferable to T1 section. speeds (ie, from 30 –120 /s). This was important
because spasticity is velocity dependent. The
Patients authors assessed the shoulder adductors, elbow
flexors and extensors, wrist flexors and extensors,
Only spastic patients capable of understanding digit flexors and extensors, prono-supinators, and
and following instructions, under the age of 20 the thumb adductor. Any fixed contracture was
years, were included in this study. Disturbances in noted, but also was confirmed in the operating
hand sensibility and mild athetosis did not preclude room under general anesthesia. Muscle stretch
surgery. To be candidates for dorsal rhizotomy, response to a single, moderate brisk speed was
patients had to have more than one muscle scoring classified according to the Ashworth scale. Re-
grade 3 (considerable increase in tone, passive cently, a modified Ashworth scale has been pro-
movement difficult) or higher on the Ashworth posed [27]. In this version, a score between grades
scale [26]. For instance, in patients with only the 1 and 2 has been introduced. It is the authors’
flexor carpi ulnaris quoted as 3, there is no indi- opinion that this new score measures something
cation for rhizotomy but rather a simple tenotomy. without clinical significance. Only patients with
Between 2000 and 2001, 20 patients underwent Ashworth grade 3 or higher need treatment.
surgery. Eleven were male and 9 were female, with By definition, athetosis is the incapacity to
an average age of 12.3 years (range 4–20 years). maintain finger position. Patients therefore were
Sixteen patients suffered from congenital hemi- asked to hold their digits extended and time was
plegia, two had traumatic brain injury, and two measured. When the position was changed, timing
were victims of stroke. The traumatic brain injury was interrupted.
and the stroke accidents occurred during the first
year of life. Athetosis was observed in five patients. Hand sensibility
Patients were assessed before surgery and at 3 and Moving light touch and pinprick was assessed
15 months after surgery. Assessment was perfor- initially in all the fingertips. Further quantification
med by two independent examiners, blind to the then was obtained with Semmes-Weinstein mono-
extent of the surgical procedure already performed filaments (Sorri, Bauru, SP) and with the moving
and to previous evaluation results. Means were two-point discrimination test. These advanced
compared by the ANOVA I method. A difference sensorial tests give comparable or even superior
of 5% in level was considered significant. No results to the more tedious shape-recognition tests
patient underwent any kind of physiotherapy in children with cerebral palsy [28].
during the postoperative period.
Grasping strength
Assessment Grasping strength was measured with a vig-
orimeter [29]. The bulb was placed between the
Range of active motion (RAM)
thumb and fingers. In older children, grasping
Initially, patients were asked to touch their strength was measured with the Jamar dynamom-
contralateral knee and then their nose, with the eter (Baseline) allowing thumb use, and pinch
690 J.A. Bertelli et al / Hand Clin 19 (2003) 687–699

strength (key pinch) was measured with a pinch- profiles. Radiographic studies were performed 1
ometer (Baseline). The best value of three week preoperatively and 3 and 15 months
consecutive attempts was recorded. postoperatively.

Functional examination Surgical technique


In general, reports of the results of surgical After general anesthesia induction, the patient
treatment of the upper limb in cerebral palsy focus was placed prone with the head in a Mayfield pin
on hand position and overall motion. Eliasson head-holder. The spinous processes of C2 and C7
et al [30] have recently included functional studies were marked and confirmed by fluoroscopy. A
in their evaluation protocol. midline incision was made across the cervical
Functional studies are related to the impact of region. A two- or three-level hemilaminectomy
the cerebral palsy on a child’s health and well being was performed. The yellow ligament was divided
and also the use of the hand in programmed and the duramere was opened. The dorsal roots
activities, allowing evaluation of fine coordination were identified and sectioned; major vessels always
and dexterity. In the former situation, several tests were preserved. The duramere was closed with
have been described [31]. From among these tests, a watertight seal and the yellow ligament sutured.
the authors have adopted the Pediatric Evaluation The removed bone chips then were replaced. The
and Disability Inventory (PEDI) [32], which con- muscle and fascial layers were reapproximated and
sists of 197 items indicating what children with the skin was closed (Fig. 1). No postoperative neck
disabilities are capable of doing and 20 items to immobilization was used.
assess the degree of caregiving assistance and
equipment modifications needed to accomplish
complex functional skills, such as eating and comm- Results
unity mobility. For the present study, only activities
Brachial plexus dorsal rhizotomy significantly
related to the upper limbs, including self-care and
relieves spasticity according to the Ashworth scale
mobility, were assessed (maximal score = 67). The
(P < 0.05). Increase in the range of motion
PEDI was developed for children with disabilities
(P < 0.05) was observed, including wrist extension
and its use is more likely to identify subtle differ-
and supination. Marked improvement in hand
ences in functional performance after treatment
dexterity and in the speed of movements
[33]. Several tests have been proposed to evaluate
(P < 0.05) was demonstrated. Grasping strength
fine motor coordination of the upper limb [34]. The
was augmented in all the patients except two
Box and Block test [35] and the Jebsen-Taylor hand
whose preoperative performance was unchanged.
function test [36] were selected because they are
In these two patients, however, pinch strength
easy to apply, are reliable, and have been validated
increased. Indeed, pinch strength increased in all
in the assessment of neurologically disabled
the patients (P < 0.05). Amelioration in daily
patients [37–39]. The Jebsen-Taylor hand function
activities measured by the PEDI test was a con-
test consists of seven activities that are timed:
stant (P < 0.05; Table 1). All the patients and
writing, turning over 12  7-cm cards (simulating
parents were satisfied with surgery and certainly
page turning), picking up small common objects,
would do it again. Several home activities became
simulated feeding, stacking checkers (to assess fine
possible after surgery, such as video game playing,
motor coordination), picking up large, light ob-
shaving of the contralateral axilla, deodorant
jects, and picking up large, heavy objects. Not all
application, eating, drinking, turning the lights
patients are able to perform all these activities.
on and off, and opening doors. Before surgery,
In the Box and Block test, patients are asked to
athletic patients were unable to keep their fingers
pick up a block and place it in an attached com-
extended for more than 3 seconds. Fifteen months
partment. The number of blocks moved is counted
after surgery (Figs. 2 and 3) they were able to
over a period of 15 seconds. Before assessment in
retain extension for more than 15 seconds
the Jebsen-Taylor and Box and Block tests,
(maximal time counted).
children underwent two training trials.
Differences between data collected at 3 months
Radiographic studies of the cervical spine and at 15 months postoperatively consisted
mainly of improvement in dexterity and in
Radiographies of the cervical spine were ob- functional activities. Recurrence of spasticity was
tained in AP view, neutral, and flexion–extension not demonstrated. When standing or walking,
J.A. Bertelli et al / Hand Clin 19 (2003) 687–699 691

Fig. 1. (A) Left C5–C7 hemilaminectomy. The duramere (D) has been opened exposing the dorsal roots (DR). The
dentate ligament (DT) separates the dorsal roots from the anterior roots. (B) C5–C8 dorsal roots have been divided.
Vessels alongside the dorsal roots have been preserved. (C) The duramere has been closed and the yellow ligament has
been sutured (arrow). (D) The removed bone chips have been replaced. (E) The surgical incision covered by an adhesive
dressing.

elbow flexion was abolished in all patients except even in a four-root section. Initially, several
one. This patient was operated on again and the patients complained about numbness, but this
biceps motor branch was sectioned. This surgery gradually disappeared. Fifteen months after sur-
was successful and active supination and elbow gery, numbness persisted in only three patients.
flexion were preserved. Deafferentation pain and complete hand anesthe-
Contractures were documented in 10 patients sia were never observed. When two roots were
before surgery. The shoulder was never involved sectioned, preoperative sensibility was unchanged.
and the elbow was involved in three patients. In When three roots were sectioned, fine sensibility
only one patient was an elbow contracture (ie, two-point discrimination less than 5 mm) was
important (90 of flexion). In the remaining preserved. When four roots were sectioned,
patients, the elbow flexion contracture was less sensibility perception decreased, but two-point
than 30 . The wrist and digital flexors were affected discrimination persisted in all patients except
more frequently. Ten patients are now candidates one. In this patient, two-point discrimination was
for tendon lengthening or transfer (Table 2). no longer possible in digits III and IV. Three
Surgical complications did not occur. None of patients had their sensibility improved after
the patients lost any movement or ended up with surgery (Table 3). Preoperative sensibility deficit
an ataxic limb. Sensibility was largely preserved, did not influence functional results.
Table 1
692
Patient information and results of Ashworth, PEDI (Pediatric Evaluation and Disability Inventory), B-Block (Box and Block test), J-Taylor (Jebsen-Taylor hand function
test), grasp strength, pinch strength, and RAM (Range of Active Motion) assessments
Case Age Sex Side Rhizotomy Ashworth PEDI B-Block J-Taylor Grasp Pinch RAM
1 19 F R C5–7 SA 1/0, EF 4/2, EE 3/0, P 2/0, 63/75 4/6 102/14 9/20 5/13 Normal/normal
WF 4/0, DF 3/0, TA 2/0
2 12 M L C5–7 SA 3/0, EF 4/1, EE 4/1, P 5/2, 54/60 4/5 25/1a 10/16 3/5 SA 145/170, S 10/80, WE 30/70
WF 4/0, DF 3/0, TA 1/0
3 11 M R C5–8 SA 2/0, EF 3/0, P 5/2, WF 4/0, DF 4/0 56/67 6/8 20/11a 8/10 3/4 S 0/20
4 14 M R C5–7 EF 4/0, EE 4/0, P 5/1, WF 3/1 60/64 6/8 58/33 16/16 7/10 SA 130/170, EE 160/175,
S 80/140, WE 50/70
5 4 F L C6–8 EF 4/0, EE 2/0 P 3/0, WF 3/0, DF 2/0 54/67 4/6 99/15 100/140 — S 0/30, WE 0/30
6 9 F R C5–8 EF 4/1, P 4/1, WF 4/1, DF 4/1 53/66 3/6 33/14a 2/10 0.8/3 S 0/60, WE 0/40
7 5 F R C5–8 EF 3/2, EE 4/1, P 3/1, WF 3/1 52/67 3/5 31/13a 60/95 S 0/30, WE 0/10
8 10 M R C5–8 SA 2/0, EF 5/0, P 4/0, WF 4/0, DF 4/0 54/67 3/8 45//22 75/120 1.5/3.5 SA 120/170, EE 45/160, S 30/90,
WF 30/60, WE 10/45
9 15 M L C6–8 EF 3/1, EE 2/1, P 2/1, WF 5/2, DF 2/0 50/65 4/5 88/25 9/13 — EE 100/140
10 4 F L C5–8 SA 2/0, EF 4/0, P 4/1, WF 2/0 53/67 4/8 99/14a 65/120 0.8/2 SA 140/180, EE 110/150,
S 0/20, WE 0/40
11 20 M R C7–8 P 3/0, WF 3/0, DF 3/0 62/66 4/8 62/27 23/26 — S 20/130, WE 0/10
12 16 M R C5–8 SA 2/0, EF 4/2, P 4/2, WF 4/2, DF 4/2 54/57 3/4 88/65 11/13 — SA 60/120, EE 0/120, EF 0/120
13 6 F R C5–8 EF 3/0, P 3/0, WF 3/0, DF 3/0 53/62 4/6 87/26a 65/130 — S 0/80
14 16 M R C6–8 P 4/2, WF 4/2, DF 4/2 60/64 4/6 58/36 22/26 4/5 WE 0/30
15 16 F L C5–8 EE 4/0, EF 4/0, P 4/0, WF 4/0, DF 4/2 61/66 3/4 41/35 60/80 — EE 110/140, S 0/50, WE 0/70
16 20 F L C7–8 WE 4/0, DF 4/0 51/61 3/4 87/55 65/75 — S 0/65
17 14 M R C5–8 EF 3/0, EE 2/0, P 4/2, WF 4/2, DF 4/0 46/66 4/6 37/14b 1/11 2.2/4 SA 140/180, S 10/90, WE 0/20
18 8 M L C7–8 WF 4/2, DF 4/0 46/56 3/5 55/36 65/85 — WE 0/30
J.A. Bertelli et al / Hand Clin 19 (2003) 687–699

19 — — L C7–8 EF 4/0, P 4/2, WF 3/0, DF 3/2 62/66 4/6 45/26 120/160 — WE 0/20
20 20 M L C5–8 SA 2/2, EF 4/2, P 4/2, WF 2/2, DF 2/2 62/67 4/8 66/25 20/20 5/6 P 120/160
Ashworth, SA (shoulder adductors), EF (elbow flexors), EE (elbow extensors), P (pronators), WF (wrist flexors), DF (digital flexors). 0–5 are the degrees of spasticity
according to Ashworth scale.
B-Block, values refer to transposed blocks.
J-Taylor, values are expressed in seconds. a ¼ one, b ¼ two new tasks regained.
Grasp, values less than 30 are expressed in kilograms and were measured with the Jamar dynamometer. Values greater than 30 are expressed in mm Hg and were measured
with the vigorimeter. The patients were unable to press the Jamar dynamometer because of weak grasp.
Pinch, values in the pinch column are expressed in kilograms.
RAM, SA (shoulder abduction), EE (elbow extension), S (supination), P (pronation), WE (wrist flexion), WE (wrist extension). The values following characters are the
results of goniometry and are expressed in angular degrees ( ).
Test results are presented as pre/post operative values, with postoperative evaluation made at 15 months.
J.A. Bertelli et al / Hand Clin 19 (2003) 687–699 693

Fig. 2. (A) Preoperative view of the hand in case 6. When trying to grasp, the digits flexed before reaching. There is no
thumb extension. (B) Fifteen months after surgery. Full digital extension is observed. Contact with the object did not
induce uncontrolled digital flexion. (C) More appropriate grasping with the object placed in the palm of the hand.
694 J.A. Bertelli et al / Hand Clin 19 (2003) 687–699

Fig. 3. (A) Preoperative view of the hand in case 11. (B) Postoperative view.

Greater improvement in function with preserva- noted by a higher score obtained in the Box and
tion of sensibility and more natural use of the Block and Jebsen-Taylor tests, which reflects
operated hand was observed in patients younger progress in dexterity and speed of movements.
than 9 years of age. None of these patients Goniometric studies point to a significant gain in
presented with digital or wrist contractures. amplitudes, especially prono-supination and wrist
No abnormalities in the radiographic studies extension. Grasping and pinch strength were
were noted. augmented. Elbow flexion during walking was
abolished. There was a marked improvement in
Discussion the performance of daily activities, expressed in the
PEDI test results and parents’ opinions according
Brachial plexus dorsal rhizotomy largely re- to their answers in questionnaires. Changes were
duced spasticity in all of the authors’ patients. observed immediately after surgery, excluding
There was a significant improvement in function amelioration caused by CNS maturation. Func-
tional improvement, however, was greater in late
Table 2 controls. Contractures were noted only after 9
Contractures, athetosis, and possible candidates for years of age, mainly in the pronator and wrist and
orthopaedic surgery digital flexors. Athetosis improved in all cases.
Contractures The results observed here demonstrate that
Orthopedic
Case EF P W D Athetosis surgery
dorsal rhizotomy not only addresses positive signs
of upper motor neurone syndrome, but also
1 X negative signs such as muscle weakness.
2 X X
3 X X Age for surgery
4 X X X
5 Nelson and Ellemberg found that half the
6 X children with a diagnosis of cerebral palsy during
7 the first year of life lost the motor signs of upper
8 X X X motor neurone syndrome by the seventh year [40].
9 X X X Seven years or later therefore has been postulated
10 as the best age for surgical treatment [41]. The
11 X X X
diagnosis of cerebral palsy, however, should be
12 X X X X
confirmed by the second year of life [42]. It is
13 X
14 possible that several of Nelson and Ellemberg’s
15 X X X patients did not have cerebral palsy. Slight
16 X X resolution of spasticity is seen in patients of 2–4
17 X X X X years of age; however, by the age of 6–9 years,
18 X fixed contractures develop [43]. Spastic muscles do
19 X not grow normally and permanent muscle con-
20 X tractures, especially of the pronators and the wrist
EF, elbow flexion; P, pronation; W, wrist flexion; D, and finger flexors, are the result of the rapid
digital flexion; X = present. growth phases [44]. When established, these
J.A. Bertelli et al / Hand Clin 19 (2003) 687–699 695

Table 3
Fingertip sensibility after brachial plexus dorsal rhizotomy
Monofilaments (gr) Moving 2-point (mm)
Case Pre Post Pre Post Numbness
1 0.05 0.05 3 4 II
2 0.05 0.05 3 3
3 0.05 0.05 4 5
4 0.05 0.05 3 I–IV(3), V(4)
5 2.00 0.2 N/A N/A
6 0.05 I–III(0.05), IV–V(2) 3 I–III(4), IV–(6)
7 N/A N/A N/A N/A
8 0.05 I–IV(0.05), V(0.2) 3 I–IV(3), Va
9 I, V(4), II–IVa I,V(4), II–IVa N/A N/A
10 N/A 0.20 N/A N/A
11 0.05 0.05 6 I(7), II–IV(6)
12 0.20 0.20 8 8
13 2.00 2.00 N/A N/A
14 0.05 0.05 3 I–IV(3), V(4)
15 I(4), II–V(2) 2.00 Ia, II–V(8) 8
16 0.05 0.05 3 3
17 I(0.2), II–V(2) I(0.05), II–V(0.2) 8 9 IV, V
18 2.00 2.00 N/A N/A
19 0.05 0.05 3 I–IV(3), V(4)
20 0.05 I,II,V(0.2), II–IV(2) 4 I(4), II, V(5), III–IVa II, IV
a
No sensorial discrimination; N/A, data not available.
All results are for digits I–V unless otherwise indicated; postoperative evaluation was performed at 15 months.

deformities need, besides dorsal rhizotomy, ten- strongly to surgery. Improvement in grasping
don lengthening. The latter procedure carries the strength might result from better wrist position.
serious risk for causing weak grasp. Physiother- Improvement in grasping strength also was ob-
apy has a questionable role in the prevention of served in the patients with wrist contractures,
fixed contractures, because to be effective, muscle however, who did not regain dorsal wrist exten-
stretching should be performed at least 8 hours sion. Experimentally, it has been demonstrated
daily [45]. Splinting is controversial and in most that dorsal rhizotomy elicits plasticity in descend-
cases discourages the use of the hand [43]. Botulin ing neurochemical pathways. For instance, dorsal
toxin might be helpful, but the benefits of rhizotomy augments descending serotonergic
functional use do not outweigh the weakness in pathways from the raphe nuclei to the spinal cord
grasping. The most notable change is the cosmetic [48]. Dorsal rhizotomy is associated with slight
benefit of reduced involuntary elbow flexion. enlargement of motor neurones and up-regulation
Moreover, in some patients functional deteriora- of BDNF and NT-3 in the ventral horn [49].
tion in the hand occurs [46]. An important finding Plasticity induced by dorsal rhizotomy therefore
of the present study is that children of lesser age could have played a role in the clinical ameliora-
present not only a more spontaneous use of the tion of the authors’ patients. Improvement in
hand but also a great functional improvement in strength could be related to resolution of co-
daily activities. Hence, based on the considerations contractions. In a preliminary study, however, the
described and the present observations, it is the authors were unable to reliably confirm this
authors’ opinion that hemiplegic children should hypothesis (unpublished observations).
preferentially undergo brachial plexus dorsal
rhizotomy between the ages of 5 and 6 years. Preservation of hand sensibility
Complete hand anesthesia was not observed in
Grasping strength
any of the authors’ patients. When two or three
In hemiplegic children, there is no increase of roots were divided, sensibility recovered to nor-
grasping strength with age [47]. The improvement mal. When four roots were sectioned, there was
in grasping observed here therefore is related preservation of sensibility on the radial side of
696 J.A. Bertelli et al / Hand Clin 19 (2003) 687–699

the hand, including sensibility to light touch. The absence of muscle feedback, subjects could volun-
ulnar digits presented a variable diminution in tarily recruit motor neurones, grade their dis-
sensibility. Gains in motor performance and use of charge, and sustain a constant level of activity.
the hand in daily activities largely outweighed the They found that inhibition of motor neurones
sensorial downgrading in every patient. Sensory during contractile fatigue was impaired. The
preservation is explained by the overlapping of afferents responsible for this monosynaptic reflex
cutaneous territory from the peripheral to the inhibition enter the spinal cord through the ventral
central nervous system. The removal of sensory and dorsal roots [58]. In the authors’ patients,
input from the limb in adult and young animals by therefore, this mechanism likely was preserved,
dorsal rhizotomy or dorsal column or spinal nerve because ventral roots were spared. Moreover, the
sectioning results in a major expansion of neigh- authors have demonstrated that muscles could be
boring body-part representation into cortical areas functionally reinnervated exclusively by motor
previously served by deafferentated limbs [50]. axons. Even in the absence of any muscle feedback,
Cusick and colleagues [51] showed that a time patients were able to learn new movements [24].
course of 1–3 days was sufficient for total re-
organization to occur, but stabilization was Transoperative rootlet electric stimulation for
achieved 8 months later. The rapid cortical dorsal rhizotomy
reorganization is probably caused by activation
Fasano et al [9] proposed the idea that not all the
of silent synapses (subthreshold input). For in-
dorsal rootlets are ‘‘spastic’’ and that the affected
stance, Li and Waters [52] have demonstrated that
ones could be identified by electrophysiologic
the cortical region of digit III (D3) in the rat
monitoring. Based on this concept, lower limb
receives suprathreshold input not only from D3
dorsal rhizotomy currently is performed after
but also subthreshold inputs from the remaining
electrical stimulation [59,60]. The electrophysio-
digits. This demonstration suggests that sub-
logic criteria for identifying abnormal rootlets,
threshold input might play a role in rapid cortical
however, have been modified frequently and the
reorganization following the loss of afferent input,
skepticism about this time-intensive monitoring
whereby neurones in the deafferentated cortex
technique has grown over the last decade [61].
could become activated by inputs from neighbor-
Moreover, Sacco et al [62] have demonstrated
ing digits. As quoted by Foerster [3], Clark,
similar outcomes between patients undergoing
Taylor, and Hildebrand have demonstrated that
selective dorsal rhizotomy with electrophysiologic
the division of four or even five adjacent thoracic
monitoring and those undergoing a random section
roots produces no apparent disturbance of sensi-
of 60% of the dorsal rootlets. These investigators
bility. Burchiel [53] states that the overlapping of
suggest that selective dorsal rhizotomy based on
sensory dermatomes is so extensive that to pro-
electrophysiologic parameters is rather nonselec-
duce just a zone of hyperesthesia, three or more
tive. It is the authors’ opinion that all dorsal
continuous dorsal roots must be sacrificed. In
rootlets related to a spastic muscle are affected
hand surgery, sensory preservation is a common
equally and should be sectioned. Partial dorsal
observation in tetraplegic patients [54]. In general,
rhizotomy, as originally proposed by Foerster [2,3],
the level of motor palsy does not correlate with the
is not only a source of incomplete relief of spasticity
level of sensory loss, which is preserved distally
but also a source of recurrence. Electrophysiologic
[55]. Finally, the contribution of the afferents in
monitoring is time-consuming and increases the
the ventral root to hand sensibility preservation
rate of anesthetic complications [63].
remains to be determined.
Spinal cord stability
Motor control after dorsal rhizotomy
In children, multiple level laminectomies may
The effects of dorsal root section on limb control cause kyphosis, anterior subluxation, and instabil-
have been extremely controversial [56]. None of the ity of the cervical spine [64]. The deformity after
authors’ patients presented limb ataxia after laminectomy is a consequence of the loss of the
surgery. In fact, motor control was largely im- posterior elements [65]. The incidence of spine
proved. Gandevia et al [57] studied hand intrinsic deformity following laminectomy is approximately
muscle voluntary activation after complete deaf- 49% [66]. The incidence of deformity has been
ferentation by anesthetic block of the ulnar nerve. decreased largely after the introduction of the
These investigators deduced that in the complete replacement laminoplasty technique [67]. In seven
J.A. Bertelli et al / Hand Clin 19 (2003) 687–699 697

cases of extended replacement laminoplasty (5 to 7 Acknowledgments


levels) with an average follow-up of 42 months,
Bergoin et al [68] found no cases of kyphosis. In The authors would like to thank the medical
fact, the complication noted in one case was staff members of the Department of Anesthesiol-
a hyperlordosis. ogy at Joana de Gusmão Children’s Hospital for
The spinal canal of the authors’ patients was their enthusiasm and expert assistance in patient
approached by a hemilaminectomy, the yellow care.
ligament was reconstructed, and the bone re-
placed. The authors’ strategy resulted in a high References
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Hand Clin 19 (2003) 701–716

Cumulative Index 2003

Volume 19
February MUTILATING HAND INJURIES, pages 1–210
May THE MUSICIAN’S HAND, pages 211–360
August ADVANCES IN OBJECTIVE ASSESSMENT OF HAND FUNCTION AND
OUTCOME, pages 361–544
November THE UPPER LIMB IN CEREBRAL PALSY, pages 545–716
Note: Page numbers of article titles are in boldface type.

A industrial, syndrome of, 325


ABILHAND, 371, 374 of instrumental musician,
instrument for, 374–375 325–326, 328–329
calibration of, 375, 376 shoulder, and hand disabilities, questionnaire
procedures for, 375 of, 366
Algodystrophy. See also Reflex Arthritis Hand Function Test, 475
sympathetic dystrophy. Arthrodesis, scapho-trapezo-trapezoid, center of
causes of, 511 rotation location and,
detection of, at 1 week, 514 414–415
diagnosis of, 513–514 cineradiography for measurement of, 413,
finger stiffness in, measurement of, 414
511–515 electrogoniometric and radiologic
following Colles fracture, 512 evaluation of, 411–419
goniometry measurements in, 513, 514 electrogoniometry for measurement of,
Amputation(s), adaptation following, 185–186 412–414
biomechanical impact of, 19–24 patients for, 412
cause for, 185 pivot points and, 415, 418
elective, length and, 185 Arthrodesis/arthroplasty, in musician,
guillotine-type, functional outcome following, 254–255
197, 198 hand therapy following, 294
in fractures of mutilated hand, 54
Arthrograph, limitations of, 511
‘‘Antagonistic’’ muscles, 218 to quantify joint stiffness, 511
Antibiotics, choice of, for mutilating hand Arthrosis, nonarticular, in swan neck deformity in
injuries, 36–37 proximal
for prophylaxis in surgery, 33 interphalangeal joint, 249, 250–251
in extremity trauma, 2–3, 34–36
side effects of, 37 Atopic dermatitis, musical instrument and, 489
Antimicrobials, in management of mutilating
injuries, 33–39
topical, irrigation with, 36 B
Baclofen, in spasticity and dystonia, 586, 587
Arm, examination of, in focal dystonia, 310
pain in, in musicians, causes of, 234 Behavioral therapy, in musicians’ dystonia, 534

0749-0712/03/$ - see front matter Ó 2003 Elsevier Inc. All rights reserved.
doi:10.1016/S0749-0712(03)00108-2
702 Cumulative Index / Hand Clin 19 (2003) 701–716

Bennett fractures, in musician, repair of, 252, 253 cingulate motor area of, 527, 529
imaging of, and assessment of fine motor
Benzhexol, in spasticity and dystonia,
control, in musicians,
586, 587
523–538
Birth defects, cerebral palsy and, 550 methods of, 523–524
Blood vessels, repair of, following mutilating of musician, auditory-sensory motor regions
injuries of hand, 5–6, of, 529, 530
8–9, 135 structural changes in, 526,
used as ‘‘spare parts’’ in mutilating injuries, 79, 527–528
83–85 regions in sensory and motor music processing,
525
Bone(s), healing of, following mutilating injuries structure of, 524–525
of hand, 135–136 supplementary motor area of, 526, 528, 529
secondary procedures on, in
mutilating injuries, 150, 151 Brass musicians, instrument-related examination
used as ‘‘spare parts’’ in mutilating injuries, 79, of, 230
83–85 British Performing Arts Medicine Trust (Britain),
Botulinum toxin, in musicians’ dystonia, 534 317, 351–353

Botulinum toxin A, in spasticity and dystonia,


585, 601 C
in upper limb spasticity in cerebral palsy, Carpal tunnel syndrome, clinical provocative tests
591–600 in, 387–391
intramuscular, clinical trials of, diagnosis of, 279
592–593 Durkan’s test in, 388, 389
effects of, 592 early, management of, 280–281
equipment for, 596, 597 evaluation of, 280
indications for, 597–598 questions in, 387
injection aftercare, 595–596 Gilliat’s test in, 388, 390
results and complications of, 597–599 hand therapy for musician in,
target muscles for, location of, 595, 596, 597 294–295
selection of, 594–595 history taking in, 387–388
treatment goals in use of, 594 local ischemia of nerve in, 279
types of, 592 management of, 243–244
Phalen’s test in, 388, 389
Brachial plexus dorsal rhizotomy. See Rhizotomy,
surgery in, 281, 282
brachial plexus dorsal.
rehabilitation following, 281
Brachial plexus palsy, obstetric, Tinel’s sign in, 388, 389
biophysical measurements in, 394 treatment of, nonoperative, 280
diagnosis and treatment of,
Carpectomy, proximal row, in wrist joint
393–394
contracture, 659, 662
evaluation techniques and scoring systems
in, 394 Cellists, hand problems of, 322–323
high spatial resolution electromyography
Central nuclei, 219
and video-assisted movement analysis
in, 393–399 Cerebellum, subcortical nuclei and,
physiotherapy in, 393–394 218–219
range of motion measurements in, 394
Cerebral cortex, 219–220
Bracing, dynamic orthotic traction, and electrical
Cerebral palsy, assessment and nonsurgical
stimulation, in spastic deformity, 603–605
management of, 545–606, 679–680
Brain, activity of, assessment of, 523–524 classification of, 560–561, 566, 567, 568
in musicians’ dystonia, 530, 531, 532, cosmesis in, assessment of, 682
533 definition of, 609
Cumulative Index/ Hand Clin 19 (2003) 701–716 703

dressing in, assessment of, 681 results of, 625–627, 628


etiology of, 547–555 surgical management of, 607–699
evaluation of sensibility in, 570 surgical management of shoulder and elbow
functional assessment in, 680 deformities in, 631–648
aided by motion laboratory studies, three subgroups of, 585
565–571 thumb in, surgical management of, 667–677
functional examination of, 561 upper limb in, functional and cosmetic
functional strength in, 575–576 outcome of surgery of, 679–685
functional testing in, 567–568 spasticity and dystonia in children,
hand deformity and dysfunction in, casting, pharmacologic management of,
splinting, and physical and occupational 585–589
therapy in, 573–584
Cervical radiculopathy, 279
hand dysfunction in, analysis and treatment in,
576–577 Child(ren), hand injuries in, types of, 121
hemiplegic, brachial plexus dorsal rhizotomy mutilating injuries in, 1, 2, 46–47, 121–132
in, 687–699 passive hand prostheses for, 186
history taking in, 566 Cineradiography, for measurement of
hygiene in, assessment of, 681 scapho-trapezo-trapezoid arthrodesis,
hypertonicity and contracture in, 574 413, 414
infant characteristics associated with, 548–550
interventions in, principles of, 573 Clarinet, ‘‘Boutonniere’’ position required on,
juvenile, evaluation of, 602 249
neuromusculoskeletal pathology of, weight of, 487–488, 489
591–592 Clarinetist, ulnar nerve damage in, hand therapy
spasticity management in, 592 following, 289
motion laboratory assessment in, 568–570
neuromuscular disorders in, 610–612 Claw nail deformity, 275
hand sensory impairment in, 612–613, 614, Cochin Rheumatoid Hand Disability
615, 616, 617 Scale, 478
physical examination in, 566–567
postneonatally-acquired, 552 Colles fracture, algodystrophy following, 512
preoperative examination in, 562–563 Complex regional pain syndrome Type I. See
prevalence of, 547 Algodystrophy.
prevention of, 552
problems of upper extremities in, Constraint-induced movement therapy, in
545–699. See also specific problems. musicians’ dystonia, 534
replacement laminoplasty in, 696–697 Contractures, and hypertonicity, in cerebral
risk factors for, antepartum, 548 palsy, 574
intrapartum, 550–551
Counter Opposition Test, of thumb, 385
preconception, 547–548
sensory examination of, 561 Cross hand transfer, for replantation in mutilating
spasticity of upper limb in, botulinum toxin A injuries, 103
in, 591–600
Cubital tunnel syndrome, diagnosis of, 279
surgery in, assessment for, 679
evaluation in, 280
data on, 681–684
local ischemia of nerve in, 279
planning for, 679, 680
treatment of, nonoperative, 280
technique of, 681
surgical, 282, 283
surgery of hand in, evaluation following, 625
goals of, 613
indications for, 616–618
preoperative evaluations for, 609–612 D
procedures for, 618–625 de Quervain tendonitis, in musician, management
prognosis in, 613–616 of, 255
704 Cumulative Index / Hand Clin 19 (2003) 701–716

Desensitization, to treat scar hypersensitivity, 138 patient selection for, 602


Dexterity, evaluation of, in impairment of hand Electrogoniometer, 6-DoF, for wrist
and wrist, 374 motion recording, 418
Diazepam, in spasticity and dystonia, 586, 587 Electrogoniometry, for measurement of
scapho-trapezo-trapezoid arthrodesis,
Digital neuritis, 284
412–414
Digit(s). See also Finger(s); Thumb.
Electromyography, high spatial resolution, in
amputation of, in musician, management of,
obstetric brachial plexus palsy, 394–396
251
in cerebral palsy, 563
fusion of, biomechanical of, 24–26
loss of, 23–24 Emergency room, management of mutilated hand
motion of, improvement following mutilating in, 51
injuries, 156–157
Epicondylitis, in musicians, 235, 284
replanted, lengthening of, 270
salvaging of, in mutilating injuries, European Association of Medicine for the Arts
6, 13 (France), 343–347
Disability of Arm, Shoulder, and Hand index, 477 Exercise(s), in treatment of musicians, 237
instrument-specific, in hand therapy for
Distal replantation, nail bed, and nail problems,
musician, 291, 292
in musicians, 259–272
protective motion, following replantation in
Dolorimeter, 512 mutilating injuries, 143–145
stability strengthening, in hand therapy for
Dupuytren contracture, hand therapy for
musician, 297–298
musician in, 293
surgery in, 253–254
F
Durkan’s test, in carpal tunnel syndrome, 388, 389 Failing Ulnar Hook Test, of ulnar palsy, 385–386
Dystonia, dopa-responsive, 587 Fatigue, lactic acid accumulation and, 217
focal. See Focal dystonia.
functional. See Focal dystonia. Federation Internationale of Medicine,
of upper limb, in cerebral palsy, 585, 586 1999 survey of, 318, 320–323
pharmacological management of, 586–587 Fibrous contracture, in cerebral palsy,
559–560
E
Fine motor control, assessment of, and brain
Edema, control of, after mutilating injuries of imaging, in musicians, 523–538
hand, 137
Finger(s), and wrist, deformity of, classification
Elbow, and shoulder, deformities of, in cerebral of, 602
palsy, surgical management of, 631–648 surgical management of, 657–665
hypertonus of flexor muscles of, Zancolli’s classification of, 560, 658
634–635, 636 as composite tissue transfer in mutilating
pain in, in musicians, 234 injuries, 79, 85
ulnar neuropathy at, 281–282 clenched fist deformity of, surgery in, 661–663,
Electric hand, for myoelectric prosthesis, 188, 664
189–190, 191 distal amputation of, management of, 261
extension of, scale of, 384
Electric hook, on wrist level disarticulation
flexion of, scale of, 382–384
prosthesis, 187, 189, 190
flexor tendon surgery of, methods of
Electrical stimulation, in management of spastic assessment of, 496
deformity, 601–606 for use in mutilating injuries, 75
dynamic orthotic traction bracing and, function of, analysis of, 421–430
603–605 dynamic measurement protocol in,
equipment for, 602–603 425–426
Cumulative Index/ Hand Clin 19 (2003) 701–716 705

finger movement evaluation equipment Flexor pollicis longus repair, assessment of, 501
for, 424–425
Flexor pronator slide, in pronation of forearm,
grip and pinch strength equipment for,
651–652
424
hand assessment protocol in, 425 Flexor tendon, assessment of function of, after
maximal isometric contraction primary tendon repair, 495–503
equipment for, 422–424 Flute player, hyperextended wrist in, 237
measurement instruments for, 426
preliminary measurements for, 426–429 Flutist, musician’s cramp in, 531
objective analysis of, 421–430 Focal dystonia, brain imaging and fine motor
fusion of, 24–25 control, in musicians, 523–538
giant cell tumor in pulp of, 248 characteristics of, 530
hyperextensible, excessive extrinsic muscle in musicians. See Musicians’ dystonia.
activity in, 235 musicians at risk for developing,
index, importance of, 22 530–531, 532
ray, elective loss of, 22–23 neuroanatomic and neurophysiologic
individual, functional contribution of, 1 background of, 523–525
long and ring, central ray deletion in, 23 of musician(s), 303–308, 318–320
microsurgical reconstruction of, characteristics of, 303
osteo-onychocutaneous flap diagnosis of, 306
for, 275 examination of instrument in, 311–312
multiple, amputation of, toe transplantation frequency, age, sex, and localization of,
for, 169–171, 173 305–306
multiple replantation of, in mutilated hand, in psychological problems, 305
102, 109–111 onset and symptoms of, 304
nonreplantable, nail salvage in, physical and neurologic examination in,
263–264 304–305
passive prosthesis for, 180–181 rehabilitation in, 309–316
permanent impairment of, evaluation of, 2 four parts of, 313–315
reconstruction of, toe-to-hand transplantation physical, 312–313
for, 169–171, 172 physical assessment for, 309–311
single, amputation of, toe transplantation for, treatment of, 306–307
169, 172 results of, 307–308
small, flexion by, 23 symptoms of, 531
stiffness in, in algodystrophy, measurement of, treatment of, 534–536
511–515 outcome of, 536
stump of, coverage of, 268
transverse amputation of, remodeling of nail Foot, surgical anatomy of, toe-to-hand
in, 268 transplantation and, 167
volar oblique amputation of, nail-recession Forearm, examination of, in focal dyatonia,
flap technique in, 268–270 310–311
Finger fillet flap, lengthening of, 76 hand and, as source of reusable parts in
mutilating injuries, 80–81,
Fingertip, amputation(s) of, 260 85–86
management of, 261–263 pronation of, classification of, 651
‘‘cap’’ composite graft, in mutilating injuries, flexor aponeurotic release in, 651
74, 75–78 flexor carpi ulnaris transfer in, 653–654
injuries to, management of, 251
replantation of, 261–263 Forearm
flexor pronator slide in, 651–652
Flexor aponeurotic release, in pronation of pronator quadratus release in, 650–651
forearm, 651 pronator teres release in, 650
Flexor carpi ulnaris transfer, in pronation of pronator teres rerouting in,
forearm, 653–654 652–653
706 Cumulative Index / Hand Clin 19 (2003) 701–716

Forearm (continued) Grip, directional, 18


surgical management of, hook, 18, 19
649–655
Grips plus action test, of hand, 385
complication of, 650, 652
postoperative treatment in, 654 Growth restriction, cerebral palsy and, 549
procedures for, 650–654 Guitarist(s), barrée function of,
supination of, procedures improving, 649, 650 examination of, 229
Foucher’s tension band nail synthesis, distal amputation of thumb of, management
266, 267 of, 276–277
examination of, 228–229
Fracture(s), complex, in mutilated
hand problems of, 322
hand, 53
inclination and torsion of guitar and,
healing of, biomechanic evaluation of, 366
assessment of, 229
in musician, repair of, 252
musculoskeletal problems in, 236
in mutilated hand, amputation in, 54
over-tensioning of strings and, 488
cases illustrating, 54–57, 58, 59
plucking techniques of, assessment of, 229
management of, 5, 7–8, 52
rehabilitation following, 57–59, 138–140
repair and reconstruction in, H
53–54 Hand(s), and forearm, as source of reusable parts
treatment of, and revascularization in, 52 in mutilating injuries, 80–81, 85–86
malunited, in musician, repair of, and wrist. See Wrist, and hand.
252–253 cross transfer of, for replantation in mutilating
simple, in mutilated hand, 52–53 injuries, 103
Free flaps, in mutilating hand injuries, electric, for myoelectric prosthesis, 188,
142–143 189–190, 191
essentials for, 17
Functional dystonia. See Focal dystonia.
examination of, in focal dystonia, 311
fisted with no wrist flexion or extension, in
cerebral palsy,
G 581–582
Ganglionectomy, hand therapy for musician function of, assessment instruments
following, 294 of, 472–474
German Society for Music Physiology and objective single measures of,
Musician’s Medicine (Germany), 474–475
347–350 prerequisites for, 573
tests of, 475–476
Giant cell tumor(s), in musician, management of, grips plus action test of, 385
254 injuries of, in children, types of, 121
in pulp of finger, 248 metacarpal, reconstruction of, toe
Gilliat’s test, in carpal tunnel syndrome, 388, 390 transplantation in, 171, 174
mutilating injuries of. See Mutilating injuries.
Glasgow Pain Questionnaire, 450–451 of musician. See Musician(s),
Gonimeter, to measure hyperlaxity in proximal hand(s) of.
interphalangeal joint, 232 basic physiologic notions of,
215–221
Goniometer, hand, 512 examination of, 225–226
Goniometry, for measurements in algodystrophy, ‘‘functional disorders’’ of, 215
513, 514 partial, passive prosthesis for,
180–181
Grap Ability Test, 476
prehension of, 17
Grasp, power, 18, 19 problems of, in musicians, dystonia as,
span, 18, 19 318–320
Cumulative Index/ Hand Clin 19 (2003) 701–716 707

hypermobility, 317–318 in non-specific wrist and hand pain,


in old injury, 317 295–296
prevention of, 317–324 treatment goals for, 297–298
prostheses for. See Prosthesis(es). in thumb carpometacarpal joint arthritis,
reflex sympathetic dystrophy of. See Reflex 295
sympathetic dystrophy. instrument-focused rehabilitation in,
replants from, for replantation in mutilating 289–290
injuries, 103, 113, 114 instrument modification in, 293
sensory impairment in, in neuromuscular instrument-specific exercises in, 291, 292
disorders in cerebral palsy, 612–613, 614, instrument-specific manual therapy in,
615, 616, 617 291–292
seven basic maneuvers of, 17–18 maintenance of musical fitness in, 292
surgery of, in cerebral palsy. See Cerebral multidisciplinary approach to, 292–293
palsy, surgery of hand in. proprioception retraining in, 298
in infantile spastic hemiplegia, 609–629 range of movement for musical position
outcome studies in, 457–458 in, 288
outcomes analysis in, 431–436 rehabilitation using instrument, 290–291
data collection process in, 433, 434 sensory re-education in, 289
measurement instrument for, 434–435 splinting of hand in, 299–300
questionnaire(s) for, choice of, stability strengthening exercises in,
433–435 297–298
examples of, 434 strengthening in, 289
steps for conducting, 431–433 treatment goals in, 287
transplantation of, assessment of functional wound, scar, edema, and pain management
outcome in, 505–509 in, 287
Chen’s score for, 507, 508
Harpists, musculoskeletal problems in, 236
Ipsen score for, 508
Tamai’s score for, 508 Hematoma, of nail bed, 265–266
evaluation chart in, 506–507 Henneman’s principle, ‘‘unit recruitment’’ and,
trauma to, biomechanics and, 17–31 216–217
classification of, 19
Hook, electric, on wrist level disarticulation
Hand posture, and hand therapy for musician, prosthesis, 187, 189, 190
298
Hypermobility, as problem in musicians, 317–318
Hand therapy, for musician, 287–301
assessment of bony alignment in, 296 Hypertonicity, and contracture, in cerebral palsy,
assessment of muscle strength in, 296–297 574
early return to modified playing and, Hyponychium, 259
288–289
early return to playing and, 291 Hypoxia, intrapartum, cerebral palsy and, 551
following arthrodesis and arthroplasty,
294
I
following ganglionectomy and benign
tumor removal, 294 Infection, antepartum, cerebral palsy and, 548
following surgery and open trauma, 287 as cause of loss of salvaged limb, 196
following ulnar nerve decompression, 295 Infertility treatment, cerebral palsy and, 547–548
good practice habits and, 290, 298–299
Injuries, mutilating. See Mutilating injuries.
hand posture and, 298
in carpal tunnel syndrome, Instrument, adjustments to, 489
294–295 atopic dermatitis and, 489
in degenerative conditions, 293 musculoskeletal pain related to, locations and
in Dupuytren contracture, 293 mechanisms of, 234–236
in hyperlaxity, 296 size and shape of, for physique of musician,
in muscle imbalance, 296 487
708 Cumulative Index / Hand Clin 19 (2003) 701–716

Instrument (continued) resting posture of, in cerebral palsy,


weight and tension of, for physique of 557–558
musician, 487–488 spastic and dystonic, in children with
cerebral palsy, pharmocologic
International Classification of Functioning,
management of, 585–589
Disability, and Health, dimensions of, 371–372
spasticity of, in cerebral palsy, botulinum
in outcome evaluation of hand and wrist,
toxin A in,
371–378
591–600
Ischemia, in mutilating injuries, functional
outcome and, 198
M
Malunion/nonunion, in mutilating injuries,
J secondary procedures in, 150, 151
Jamar Hand Dynamometer, 421
Manipulation, and dexterity, in cerebral palsy,
Jebson Hand Function Test, 476 576
Joints, laxity of, in musicians, 235 Memory, stages of, 220
range of motion of, in cerebral palsy, 560–561
secondary procedures on, in mutilating Metacarpal boss(es), in musician, management of,
injuries, 156–159 254
stiffness of, in algodystrophy, 511 Michigan Hand Outcomes Questionnaire, 477
treatment following mutilating injuries,
Microsurgical reconstruction, secondary, in nail
140, 141
problems in musicians, 273–278
used as ‘‘spare parts’’ in mutilating injuries,
79, 83–85 Misuse syndrome(s), 328
Moberg pickup test, in impairment of hand and
K wrist, 373–374
Keitel Function Index/hand Functional Index,
Motion analysis, video-assisted, in obstetric
475
brachial plexus palsy, 396–399
Keyboard players, musculoskeletal problems in,
Motion laboratory studies, for functional
236
assessment in cerebral palsy, 565–571
purpose of, in cerebral palsy,
L 565–566
Laminoplasty, replacement, in cerebral palsy, Motor action, study of, elementary level of,
696–697 215–217
L-Dopa, in spasticity and dystonia, 586, 587 Motor tract, 215, 216
Limb, survival of, and limb function, 195–196 Motor unit(s), connections of, 217–218
upper, clinical evaluation of, in cerebral palsy,
557–563 Movement disorders, motion analysis in,
evaluation of spasticity of, in cerebral palsy, 396–397
558 Muscle fatigue, 327
functional and cosmetic outcome of surgery
of, in cerebral palsy, 679–685 Muscle strength testing, in impairment of hand
general assessment of, in cerebral palsy, and wrist, 373
557–564 Muscles, response to excitation, 217
in cerebral palsy, surgical management of
Muscular fiber, 215, 216
shoulder and elbow deformities in,
631–648 Musculoskeletal pain, in musicians, prevention of,
joint function of, clinical evaluation of, 239
379–386 treatment of, 236–238
motor assessment of, in cerebral palsy, related to instrument, locations and
558–559 mechanisms of, 234–236
Cumulative Index/ Hand Clin 19 (2003) 701–716 709

Musculoskeletal pain syndrome, 233–234 hand symptoms in, 224–225


hand therapy for. See Hand therapy, for
Musculoskeletal problems, in instrumental
musician.
musicians, 231–239
highly trained, loss of manual coordination in,
in string players, 235–236
530–531
Music performance, neurophysiology of, incisions in, planning and location of, 247
525–527 injuries of, determination of structures injured
Musical instrument. See Instrument and specific in, 242
instruments. instrumental, musculoskeletal problems in,
231–239
Musician(s), anatomic compromise in, adjustment managers of, prevention of hand problems by,
to, 249, 250 323
anatomic reconstruction in, 248 medical care of, national organizations
brain of, auditory-sensory motor regions of, involved with, 343–353
529, 530 misuse, overuse, and repetition in,
structural changes in, 526, controversies surrounding,
527–528 325–329
carpal tunnel syndrome in. See musculoskeletal pain in, prevention of, 239
Carpal tunnel syndrome. treatment of, 236–238
chronic pain in, 238–239 musculoskeletal problems in, categories of, 231
distal replantation, nail bed, and nail problems evaluation of, 232
in, 259–272 frequency of, 231
doctor communicating with, 223 risk factors for, 232–234
examination and interface with, nail problems in, secondary microsurgical
223–230 reconstruction of, 273–278
exercise in treatment of, 237 nerve compression syndrome in, management
focal dystonia, brain imaging, and fine motor of, 243, 279–286
control in, 523–538 nontrauma conditions of, surgical
hand(s) of, acute trauma to, surgical considerations in, 253–254
management in, 243 pain as symptom in, 225
and visual and technical motion analysis performance-induced problems in, 231
studies of, 226 physical therapy in treatment of, 235
basic physiologic notions of, practice habits of, assessment of, 224
215–221 special operative considerations in, 247–258
biomechanic examination of, 226 surgery in, contraindications to,
clinical evaluation of, 484 242–243
extremity and, 484 rehabilitation and return to play following,
interface in, 484–486 249–253
evaluation of instrument and, 486–488 results of, 256–257
examination of, 225–226 surgical assessment of, 241–245
function and outcome assessment of, trauma to, requiring surgery, incidence of, 247,
483–493 248
‘‘functional disorders’’ of, 215
injuries of, objective assessment of, 491 Musician(s)
instrument-related examination of, results of, 249
226–230 with hand problems, history taking in, 223–224
investigation of, special techniques of, with pain, by instrument and gender, 233
489–490 Musician’s cramp. See Focal dystonia.
musical instrument digital interface and,
Musicians’ dystonia, 530–531
226
assessment of brain acitivity in, 530, 531, 532,
physical examination of, 242
533
surgery on, planning of, 490–491
diagnosis of, 490
treatment of, advances in,
maladaptive plasticity and, 531–534
490–491
710 Cumulative Index / Hand Clin 19 (2003) 701–716

Musicians (continued) treatment views and, 195


musicians at risk for developing, outcomes in, 51
530–531, 532 pain in, 45–46
musician’s cramp in, examples of, 530, 531 patient evaluation in, 73–75
psychologic support in, 535–536 perionychium for reconstruction in, 75, 78–80
treatment of, 534–536 physical examination in, 3–4
principles of, and management of, 1–15
Mutilating injuries, antimicrobial management of,
psychological aspects of, 41–49
33–39
psychological intervention strategies in, 44–45
bacteriology of, 33–34
psychological responses to, 42–43
bones, tendons, nerves, vessels, and joints used
rehabilitation process for, 133
in, 79, 83–85
replantation in. See Replantation, in mutilated
‘‘cap’’ composite tip graft in, 74, 75–78
hand.
complex, management of, 6–9,
secondary procedures following, 149–163
10–13, 14
early care influencing, 150
crush, outcomes after, 196
involving nerves, 155–156
debridement and irrigation in, 4–5
on bone, 150, 151
electrical, outcomes after, 196, 197
on joints and tendons, 156–159
emergency room management of, 51
on soft tissue, 151–152
etiology of, 51, 63
on thumb, 159–161
finger as composite tissue transfer in, 79, 85
requirements for hand function and, 13, 150
finger for use in, 75
to release scar, 152–155
fracture fixation in. See Fracture(s),
skin harvesting in, 76–78, 80–83
in mutilated hand.
soft tissue coverage in. See Soft tissue coverage,
hand and forearm as source of reusable parts
in mutilating injuries.
in, 80–81, 85–86
special issues in, 45–47
healthy adjustment of, promotion of, 43–44
stabilization of patient in, 1
history taking in, 1–3
therapy following, 133–148
in children, 1, 2, 46–47, 121–132
early phase (protective), 133–134
delayed treatment of, 128–130, 131
healing process and, 134–136
initial management and preoperative eva-
intermediate phase (mobilization), 134
luation in, 121–124
late phase(strengthening), 134
operative procedure in, 124–125
use of ‘‘spare parts’’ in, 73–87
replantation and revascularization in,
125–126 Myotatic reflex, 218
soft tissue coverage in, 126–131
initial surgery of, 52
injury-related issues in, 41–42 N
limb of hand salvage in, factors influencing, Nail(s), anatomy of, 260
194–195 Foucher’s tension band synthesis of, 266, 267
management of, and principles of, 1–15 injury(ies) of, basic principles of management
factors influencing, 63 of, 264
immediate, 1, 2, 3, 4–5 isolated, 264–268
reconstructive algorithms in, 63–65 techniques to save nail in,
outcomes after, 193–204 260–261
age as influence on, 198 lesions of, classification of, 264
assessment of, 199 distal phalanx and, 260
clinical outcomes, 195–200 loss of, due to trauma, 275
factors influencing, 193, 196–197 microsurgical reconstruction of, free
goals of, 195 vascularized composite partial toe transfer
objective assessment of, 201–202 in, 273–276
outcomes research on, 201 free vascularized nail graft in, 273, 274
salvage of function and, 193, 194 surgical technique for, 273–276
scoring systems for, 200–201 microsurgical transfer of, 263
Cumulative Index/ Hand Clin 19 (2003) 701–716 711

problems of, distal replantation, and nail bed secondary procedures on, in
problems, in musicians, 259–272 mutilating injuries, 155–156
in musicians, secondary microsurgical used as ‘‘spare parts’’ in mutilating injuries, 79,
reconstruction of, 273–278 83–85
remodeling of, in transverse amputation, 268
Nerve compression, in musician, management of,
salvage of, in nonreplantable finger, 263–264
243
reposition-flap technique for, 263, 264
whole composite reattachment of, 263–264 Nerve compression syndrome(s), in musician, 256
incidence of, 279
Nail bed, 259
management of, in musicians,
hematoma of, 265–266
279–286
loss of substance of, 266, 268, 269
problems of, distal replantation, and nail Nerve impulse, definition of, 215
problems, in musicians, Neuritis, digital, 284
259–272
repair of, 260–261 Neurologic disorders, cerebral palsy and, 547
split-thickness graft of, 266, 269 Neuroma, management of, following mutilating
wounds of, 265–266, 267 injuries, 156
Nail complex, anatomy of, 259–260 Neuromuscular disorders, in cerebral
Nail flap, thenar, de-epithelialized, repair of, 266, palsy, 610–612
268 hand sensory impairment in, 612–613, 614,
615, 616, 617
Nail graft, free vascularized, in microsurgical
reconstruction of nail, 273, 274 Neuromuscular spindle, 218
Nail matrix, 259 Neuron, 215
lesions of, management of, 266–268, 269 Nonsteroidal anti-inflammatory drugs, in
Nail plate, soft tissues beneath, 259 musculoskeletal problems, 237–238
Nail-recession flap technique, in volar oblique
amputation, 268–270 O
Osteomyelitis, following mutilating injuries,
Nail walls, 259–260
150–151
lesions of, repair of, 268, 269
Osteosynthesis, in replantation in
Nail-recession flap technique, in volar oblique
mutilated hand, 96, 98
amputation, 268–270
Outcome study(ies). See also under specific areas
Nerve(s), delayed repair of, following mutilating
studied.
injuries, 5, 9, 155–156
initiation of, measurements and,
healing of, following mutilating injuries of
458–459
hand, 135
negotiation in, 459–460
lacerations of, in musician, repair of,
patient recruitment for,
251–252
460–461
peripheral, functional recovery of, outcome
study design and, 459
study of, 458
prospective, difficulties in conducting,
protection of, following mutilating injuries of
457–462
hand, 137–138
repair of, outcome after, Overuse syndrome(s), 233–234, 326
development of model occupational, 327, 333–335
instrument for, 464–468 classification of, diagnostic algorithm for,
new model instrument for, 463–470 335
outcome measures after, currently used, 464 nonspecific, background of,
numeric test protocol for, 466–467, 468 331–333
requirements for, 463–464 classes of, 334
problem after, 463 definitions of, 333–335
712 Cumulative Index / Hand Clin 19 (2003) 701–716

Overuse syndrome (continued) Posterior interosseous nerve syndrome, 283–284


in musicians, pathophysiology of, treatment of, 284
331–341
Pre-eclampsia, cerebral palsy and, 548
research findings in,
336–339 Pronator quadratus release, in pronation
research orientation and methodology of forearm, 650–651
for study of, 335–336 Pronator teres, release of, in pronation of
subcategories of, 334 forearm, 650
pathophysiology of, 326–327 rerouting of, in pronation of forearm, 652–653
study in musicians, 327–328
Prosthesis(es), active, 13, 185–191
patient acceptance of, 186–188
P fabrication of, 187, 189, 190
Pain, chronic, in musicians, 238–239 flexible silicone sleeve of, 189, 191
in mutilating injuries, 45–46 passive, 177–183
candidates for, 177, 178
Patient-Rated Wrist Evaluation questionnaire, distal phalanx, 183
477–478 finger, 183
Pedicle flaps, in mutilating hand injuries, 142 for child, 186
goals and expectations for, 13, 177–178, 179
Percussionists, musculoskeletal problems
master plan for treatment using, 179
in, 236
middle phalanx, 183
Performing Arts Medicine Association (USA), new material and technology
350–351 for, 180
partial hand, 180–181
Perionychium, reconstruction of, in mutilating
proximal phalanx, 183
injuries, 75, 78–80
thumb for, 181–183
Phalanx, distal, 259 types of, 179–183
and nail lesions, 260 suspension on limb, 189, 190–191
proximal, passive prosthesis for, 183 transcarpal level, with electric hand, 188,
Phalen’s test, in carpal tunnel syndrome, 388, 189–190, 191
389 voluntary closing grasping device on, 190, 191
wrist level disarticulation, electric hook on,
Pharmocology, in management of upper limb 187, 189, 190
spasticity and dystonia, in children with with semi-flexible inner socket, 188, 191
cerebral palsy, 585–589
Proximal interphalangeal joint, hyperlaxity in,
Physical therapy, in treatment of gonionmeter to measure, 232
musicians, 235 splints to support, 234
Pianist(s), brain of, 529, 530 swan neck deformity in, nonarticular arthrosis
examination of, 228 in, 249, 250–251
hand problems of, 321
musician’s cramp in, 531 R
pressure development in, ergonomic Radial tunnel syndrome, 284
considerations in, 228
musical instrument digital interface and, Radiculopathy, cervical, 279
228, 229 Radiography, in cerebral palsy, 563
weight of piano and, 488
Range of motion measurements, in impairment of
Pinch, key, 18 hand and wrist, 373
oppositional, 17–18
Reflex sympathetic dystrophy, of hand,
precision, 17
517, 518
Position tolerance, gravity-dependent, after assessment of severity of, scoring system in,
mutilating injuries of hand, 137 517–521
Cumulative Index/ Hand Clin 19 (2003) 701–716 713

classification of, 517, 520–521 surgical techniques for, 690


incidence of, 517 transoperative rootlet electric stimulation
signs and symptoms of, 517, 518, 519–520 for, 696
three-phase bone scintigraphy in, 518, 519 posterior, for spasticity, history and evolution
of, 687–688
Rehabilitation, following fractures in mutilated
hand, 57–59
following mutilating injuries, 133,
S
138–141, 142
following toe-to-hand transplantation, Scapho-trapezo-trapezoid arthrodesis. See
171–172 Arthrodesis, scapho-trapezo-trapezoid.

Repetition strain injury. See Overuse syndrome(s), Scaphoid, fracture of, 3D reconstruction of,
occupational. 405–406
unstable, evaluation of, 404, 406
Replantation, in mutilated hand, 89–119
care of amputed part for, 91, 94 Scapholunate instability, evaluation of, 404
debridement and labeling for, Scar, management of, after mutilating injuries of
92–94, 96 hand, 137
hand replants in, 103, 113, 114 palmar contracture, following crush injury,
history of, 89, 90, 91, 92 management of, 153–156
indications for, 89–91, 92, 93 release of, in mutilating injuries,
issues in, 47 152–155
multiple finger replantation for, 102, web contracture, following crush injury,
109–111 management of, 152,
operative considerations for, 153, 154
92–100, 101, 102, 103, 104
osteosynthesis techniques for, Semmes-Weinstein monofilament test, in
96, 98 impairment of hand and wrist, 373
outcomes following, 107, 199 Sensory evaluation, quantitative, in impairment of
postoperative care following, 103–105 hand and wrist, 373–374
preoperative considerations for, 91–92, 94,
Sesmoid arthrodesis, in thumb-in-palm deformity,
95
674, 675
protective motion exercises following,
143–145 Shape/Texture Identification test, 465
repair of volar structures for,
Shoulder, acquired spasticity of,
98–100, 101, 104, 105
631–634
special considerations for,
surgery in, indications for,
100–107
635–638
tendon repair for, 98, 99, 100
procedures in, 640, 642
thumb replants for, 100–102, 105–109
results of, 638–647
upper extremity replants for, 102–103,
and elbow, deformities of, in cerebral palsy,
111–112
surgical management of,
Rheumatoid arthritis, postoperative outcome in, 631–648
wrist and hand handicap and, 471–481
Shoulder
Rhizotomy, brachial plexus dorsal, anatomic as three-axes joint, 379–386
basis of, 688–689 extension of, 379
candidates for, 689, 694–695 Intercalcted Fist Test of, 382, 383
in hemiplegic cerebral palsy, scapulohumeral internal rotation and
687–699 abduction of, 634
motor control after, 696 scapulohumeral internal rotation and
pathophysiologic basis of, 688 adduction of, 634
patient assessment for, 689–690 triple point test of, 379–382
results of, 690–694 Waiter’s Test of, 382, 383
714 Cumulative Index / Hand Clin 19 (2003) 701–716

Shoulder girdle, examination of, in focal dystonia, Subcortical nuclei, and cerebellum,
310 218–219
Skin, harvesting of, in mutilating injuries, 76–78, Südeck atrophy. See Algodystrophy.
80–83
Superficialis tendon, for reconstruction of thumb,
therapy of, following mutilating injuries of
21
hand, 134–135
Swan neck deformity, in proximal interphalangeal
Skin flaps, for reconstruction of mutilated hand,
joint, nonarticular arthrosis in, 249, 250–251
65–67
Swan-neck deformity, 617, 622–623
Soft tissue, beneath nail plate, 259
pathophysiology of, 661–662, 663
damage to, in mutilating injuries, 197
surgical correction of, 623–625,
secondary procedures on, in
662–663, 664
mutilating injuries, 151–152
Swellings, in hand of musician,
Soft tissue coverage, in mutilating injuries, 63–71
management of, 254
assessments for, 4, 6–7
cases illustrating, 67–69, 70 Synapse(s), 216
distant flaps for, 65–66
free flaps for, 66–67
T
in children, 126–131
local flaps for, 65 Teachers (music), and prevention of hand
regional flaps for, 65 problems in students, 323–324
of mutilating injuries, 142–143 Tendonitis, de Quervain, in musician,
Spastic deformity, dynamic orthotic traction management of, 255
bracing and electrical stimulation in, 603–605 in musicians, 235
management of, electrical Tendon(s), extensor, injuries of, 27
stimulation in, 601–606 flexor, loss of function of, 28
Spastic hemiplegia, infantile, surgical healing of, following mutilating injuries of
management of hand in, 609–629 hand, 135
injuries of, in musician, repair of, 252
Spasticity, acquired, conditions leading to, 631, loss of, and hand function, 26–28
632–633 primary repair of, assessment of flexor tendon
management of, in juvenile cerebral palsy, 592 function after, 495–503
of upper limb, evaluation of, in cerebral palsy, Buck-Gramcko methods of assessment of,
558 499, 501
in cerebral palsy, 585, 586 current methods of assessment following,
pharmacological management of, 586–587 497–501
posterior rhizotomy in, history and evolution failure of, assessment of, 496
of, 687–688 Louisville method of assessment of, 498
Spinal axis, examination of, in focal dystonia, 310 Moiemen-Elliott method of assessment of,
500
Spinal cord, 218 partial failure of, assessment of, 496–497
Spine, assessment of, in focal dystonia, 310 Strickland methods of assessment of,
498–499
Splinting, and casting, in cerebral palsy, 574–575 TAM method of assessment of, 498
in hand therapy for musician, 299–300 repair of, for replantation in mutilated hand,
in musculoskeletal problems, 237 98, 99, 100
Splints, traction, in fractures in mutilating secondary procedures on, in
injuries, 138, 140 mutilating injuries, 156–159
secondary reconstruction of, following
Split-thickness graft, of nail bed, 266, 269
mutilating injuries, 159
String players, musculoskeletal problems in, used as ‘‘spare parts’’ in mutilating injuries, 9,
235–236 79, 83–85
Cumulative Index/ Hand Clin 19 (2003) 701–716 715

Tennis elbow, in musician, 255 insetting of transplants in, 167


intraoperative and postoperative
Tenolysis, following mutilating injuries, 157–159
complications of, 172–174
Thoracic outlet syndrome, 282–283 late complications of, 174–175
symptoms of, 283 postoperative management for, 171
treatment of, 283 preparation for, 165
Thrombophilias, cerebral palsy and, 550 recipient site preparation for, 166–167
rehabilitation following, 171–172
Thumb, adduction of, and minimal wrist flexion, second toe transplantation for,
in cerebral palsy, 576–577 168–169, 171
avulsion of nail complex of, toe transfer in, surgical anatomy of foot and, 166
273, 274 timing of, 165
carpometacarpal joint arthritis in, hand total great toe transplantation in, 167
therapy for musician in, 295 trimmed great toe transplantation in, 167–168
Counter Opposition Test of, 385
distal amputation of, whole toe transfer in, Toe transfer, free vascularized composite partial,
273–275, 276–277 in microsurgical reconstruction of nail, 273,
for passive prosthesis, 181–183 274
functional importance of, 1, 19–20 in avulsion of nail complex of thumb, 273, 274
in cerebral palsy, surgical Total Opposition Test, of thumb, 384
management of, 667–677
Trigger finger, in musician, management of, 255
level five injuries of, 21–22
level four injuries of, 21 Trihexyphenidyl, in spasticity and dystonia, 586,
level three injuries of, 20–21 587
reconstruction of, priorities of, 20
Tumors, benign, removal of, hand therapy for
toe-to-hand transplantation for, 167
musician following, 294
replants for, in mutilated hand,
100–102, 105–109
secondary reconstruction of, in mutilating U
injuries, 159–161
Ulnar nerve, decompression of, hand therapy for
Total Opposition Test of, 384
musician in, 295
Thumb-in-palm deformity, assessment of, 667
Ulnar neuropathy, at elbow, 281–282
classification of, 668–669, 670
in clarinetist, hand therapy following, 289
extrinsic release in, 671–674
in cerebral palsy, 667–668 Ulnar palsy, Failing Ulnar Hook Test of, 385–386
intrinsic release in, 670–671, 672 Upper extremity(ies), motion analysis in, 397
joint stabilizations in, 674 mutilated, replantation in, replants for,
muscles involved in, 668 102–103, 111–112
natural history of, 667 mutilating injuries of, use of ‘‘spare parts’’ in,
nonoperative treatment of, 669 73–87
sesmoid arthrodesis in, 674, 675 trauma to, antibiotics in, 34–36
surgical treatment of, 669–674 side effects of, 37
timing and staging of treatment of, 670
Thyroid disease, cerebral palsy and, 548
V
Tinel’s sign, in carpal tunnel syndrome, 388, 389
Vasculitis, focal nerve pathology in, 279
Toe-to-hand transplantation, 165–175, 194
Vibration perception thresholds determinations,
for finger reconstruction, 169–171, 172
in impairment of
for thumb reconstruction, 167
hand and wrist, 373
great toe wraparound flap, 168
harvesting of toe for, 166 Violinist(s), bow positions of, 227
in mutilating injuries, motion program bow techniques of, 227
following, 145–146 examination of, 226–228
716 Cumulative Index / Hand Clin 19 (2003) 701–716

Violinist(s) (continued) fingers and, deformity of, classification of, 602


fingering techniques of, 228 surgical management of, 657–665
hand problems in, 321–322 Zancolli’s classification of, 560, 658
instrument characteristics and, 228 flexion deformity of, and no active wrist
musician’s cramp in, 531 extension, in cerebral palsy, 578–580
shifting between positions and, 227–228 release/lengthening of spastic flexor muscles
in, 657–658
tendon transfers to augment wrist extensors
W in, 658–659, 660–661
Woodwind musicians, instrument-related treatment options in, 657, 658
examination of, 230 wrist stabilization in, 659
musculoskeletal problems in, 236 function of, evaluation of, medical
imaging-based kinematic analysis in,
Wound care, after mutilating injuries of hand, 401–409
136–137 functional motion of, requirements for, 25
Wrist, and hand, clinical evaluation of, 361 fusion of, 25–26
functional evaluation of, 361–362 ganglions of, in musician,
and outcome evaluation of, management of, 254
361–369 hyperextended, in flute player, 237
four-hundred points score, 363, 364 isokinetic and isometric dynamometry of,
recent progress in, 362–366 364–366
impairment of, dexterity evaluation in, 374 joints of, measurement of, 362
evaluation of, 372–374 limited fusions of, 25–26
Moberg pickup test in, 373–374 minimal flexion of, and thumb adduction, in
muscle strength testing in, 373 cerebral palsy, 576–577
quantitative sensory evaluation in, moderate flexion of, and active wrist extension,
373–374 in cerebral palsy, 577–578
range of motion measurements in, 373 pathology of, 401
Semmes-Weistein monofilament test in, patient-focused outcome instrument for,
373 activities of daily living and, 437–438
vibration perception thresholds construct validity of, 449–450, 451–452, 453
determinations in, 373 detection of change over time in,
non-specific pain in, hand therapy for 452–454
musician in, 295–296, 297–298 development of, 437–448
outcome evaluation of, Biometrics system frequency of responses in,
in, 361, 362 440–441, 442
health-related quality of life and, preliminary testing of, 439
375–377 recruitment of subjects for,
International Classification of 450–451, 452
Functioning, Disability, and Health response options on, 438
in, 371–378 statistical analysis in, 451
and hand handicap, postoperative outcome test-retest reliability of, 441–443
evaluation in rheumatoid arthritis, validity of, 449–455
471–481 three-dimensional imaging of, 401
computed tomography of, 401–402 three-dimensional reconstruction of, 402, 403
electrogoniometric evaluation of, 364 clinical application of, 404–407
extension with intrinsic hypertonicity, in qualitative analysis of, 404
cerebral palsy, 580–581 quantitative analysis of, 404, 405

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