|
Team Approach: Single-Event
Multilevel Surgery in Ambulatory
Patients with Cerebral Palsy
Andrew G. Georgiadis, MD
Michael H. Schwartz, PhD
Kathryn Walt, PT
Marcia E. Ward, MD
Peter D. Kim, MD
Tom F. Novacheck, MD
Investigation performed at Gillette
Children’s Specialty Healthcare,
St. Paul, Minnesota
COPYRIGHT © 2017 BY THE
JOURNAL OF BONE AND JOINT
SURGERY, INCORPORATED
JBJS REVIEWS 2017;5(8):e10 · http://dx.doi.org/10.2106/JBJS.RVW.16.00101
Abstract
» Assessment of the ambulatory patient who has cerebral palsy (CP)
involves serial evaluations by orthopaedic surgeons, neurosurgeons,
rehabilitation specialists, and therapists as well as 3-dimensional gait
analysis (3DGA). The most common subtype of CP in ambulatory
patients is diplegia, and the most common severity is Gross Motor
Function Classification System (GMFCS) Levels I, II, and III.
» Increased tone in the skeletal muscle of patients with CP can be
managed with focal or generalized, reversible or irreversible means.
One method of irreversible tone control in spastic diplegia is selective
dorsal rhizotomy. A careful preoperative assessment by a multidisci-
plinary team guides a patient’s tone-management strategy.
» Abnormal muscle forces result in abnormal skeletal development.
Resultant lever-arm dysfunction of the lower extremities creates gait
abnormality. A comprehensive assessment of gait is performed with
3DGA, supplementing the clinical and radiographic examinations for
surgical decision-making.
» Single-event multilevel surgery (SEMLS) involves simultaneous
correction of all musculoskeletal deformities of the lower extremities in
a single setting. Specialized centers with attendant facilities and
expertise are necessary. SEMLS often follows years of medical
treatment, therapy, and planning. Some procedures can be performed
with the patient in the prone position for technical ease and optimal
assessment of transverse-plane alignment.
» Objective gains in patient function are made until 1 to 2 years after
SEMLS. Most ambulatory children with diplegia undergoing SEMLS
maintain function at their preoperative GMFCS level but can make
quantifiable improvements in walking speed, oxygen consumption,
gait quality, and patient-reported functioning.
Clinical Scenario
A
10-year old girl with spastic dorsal rhizotomy at the age of 7 years with
diplegic cerebral palsy (CP) is improvement in gait and energy expendi-
evaluated for difficulty with ture (Video 1 and Video 2). The patient
walking and tripping. Medical has weekly physical therapy for lower-
history includes prematurity, low birth extremity motion and strengthening and
weight, neonatal intensive care unit stay, has follow-up with orthopaedic surgery and
ventilator support, and developmental physiatry services for serial examinations
delay. Surgical history includes selective and hip surveillance. She functions at Gross
Disclosure: No external funds were received in support of this study. The Disclosure of Potential Conflicts
of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSREV/A243).
1
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Motor Function Classification System
(GMFCS) Level I1.
Examination reveals no fixed con-
tractures, excessive internal hip rotation,
increased femoral anteversion, and in-
creased external transmalleolar axes,
suggesting external tibial torsion. Left
and right popliteal angles are 40° and
50°, respectively. With the foot in sub-
talar neutral, ankle dorsiflexion is 0° and
10° with the knees extended and flexed,
respectively. There is no spasticity in
any lower-extremity muscle group.
Cerebral Palsy and Ambulation
Cerebral palsy refers to any disorder of
movement or posture resulting from a
static lesion to the developing brain. An
upper motor neuron lesion in the motor
cortex or internal capsule may lead to loss
of lower motor neuron inhibition, which
may manifest as spasticity, clonus, co-
contraction, hyperreflexia, and loss of
selective motor control. Increased tone
and gravitational forces result in poor
longitudinal growth of skeletal muscle,
progressive contracture2, and long-bone
torsion. A lesion in the basal ganglia often
results in dystonia or complex movement
disorders. Despite these patterns, the
neuroanatomical basis for movement
disorders is exceedingly complex.
Gross motor milestones are gener-
ally delayed in patients with CP, and
prolonged infantile reflexes negatively
affect walking potential3. Attaining in-
dependent sitting balance by the age of
2 years predicts ambulatory status by the
age of 8 years4,5. The GMFCS (Fig. 1) is
the most widely accepted and validated
scale that is used to categorize motor
function and ambulatory ability in pa-
tients with CP6,7. Since its inception,
this scale has been extended and revised
for 4 age groups: 2 to ,4 years, 4 to
,6 years, 6 to ,12 years, and 12 to 18
years1. Longitudinal observation sug-
gests that children who are classified as
GMFCS Level I to IV reach 90% of
gross motor development by the age
of 4 to 5 years8. The most common
subtype of CP in ambulatory patients is
diplegia, and the most common severity
is GMFCS Levels I, II, and III.
2 AUGUST 2017
Evaluation of Orthopaedic
Deformity in Ambulatory Patients
with CP
Gait abnormality in ambulatory patients
with CP results from the underlying
neurological insult. The primary deficits
are loss of selective motor control,
weakness, spasticity, and reduced equi-
librium. Secondary effects are muscle
stiffness and musculotendinous con-
tracture, primarily affecting biarticular
muscles. With growth and lengthening
of skeletal segments, tightness can
progress to fixed contracture. Abnormal
muscular forces on the growing skeleton
induce abnormal osseous development,
resulting in long-bone torsion and foot
deformity. Despite tone management,
most ambulatory spastic diplegic pa-
tients develop fixed osseous deformity,
termed lever-arm dysfunction, which can
lead to the recommendation for ortho-
paedic correction. Lever-arm dysfunc-
tion is a generic term for disruption of
a muscle-joint complex as the result of
an ineffective lever arm (e.g., femoral
torsion, tibial torsion, planovalgus foot),
resulting in functional weakness and
diminished power generation9.
The natural history of gait in am-
bulatory patients with untreated CP is
often one of progressive deterioration10,
although there is tremendous heteroge-
neity. Ambulatory children often expe-
rience worsening of hip, knee, and ankle
kinematics as well as decreased joint
motion with age.
A so-called diagnostic matrix in-
corporates all inputs (medical history,
physical examination, imaging, 3-
dimensional gait analysis [3DGA]) to
inform decision-making to optimize
patient walking (Fig. 2-A)11. Most
ambulatory children with CP undergo
serial evaluations of gait during devel-
opment with input from orthopaedists,
physiatrists, therapists, mechanical en-
gineers, and, occasionally, neurosur-
geons (Fig. 2-B).
Patient Assessment
The physical examination of an ambula-
tory child with CP has been described in
detail12. A data-collection tool organized
by anatomical location, soft-tissue defor-
mity, osseous deformity, and neurological
examination consolidates information
into 1 location (Fig. 3). Observational gait
analysis will allow the examiner to assess
assistive devices, walking pattern, and foot
and knee positioning. Areas of further
clinical attention are isolated for detailed
evaluation. Muscle strength is assessed
manually in all lower extremities with a
5-point scale13. Selective motor control is
recorded for each muscle group on the
basis of the patient’s ability to isolate vol-
untary movements9. Abnormal tone can
be spastic (velocity-dependent resistance
to passive elongation), dystonic (activity at
rest, uncoordinated movements distant to
the desired movement), rigid, or mixed.
Grading scales are available to differentiate
spasticity14 from dystonia15.
Static assessment of joint motion
depends on proper limb positioning
during testing, which is critical for
identifying a contracture. The Thomas
test assesses hip-flexor tightness when
the anterior superior and posterior su-
perior iliac spines are vertically aligned.
Unilateral and bilateral popliteal angle
measurements can differentiate true
from functional hamstring contracture
and can provide information about
contralateral hip-flexion contracture16.
The Silfverskiöld test differentiates gas-
trocnemius and soleus tightness17, and
the Duncan-Ely test assesses tightness
of the biarticular rectus femoris9.
Transverse plane osseous defor-
mity of the lower extremities is best
assessed with the patient in the prone
position, with the findings being con-
firmed or controverted on the basis of
3DGA and imaging. Femoral antever-
sion can be statically assessed with use
of the trochanteric prominence test18,
although obesity, varied trochanteric
geometry, or prior surgery can lead to
underestimation of anteversion19. We
assess tibial torsion with 3 methods: the
prone thigh-foot axis, the bimalleolar
axis, and the second-toe test12. Posi-
tioning of the hindfoot, midfoot, and
forefoot in both weight-bearing and
non-weight-bearing positions is docu-
mented. The talonavicular joint must
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Fig. 1
The Gross Motor Function Classification Scale for patients 6 to 12 years of age, detailing independent ambulatory and movement
ability. (Reproduced with permission, © Bill Reid, Kate Willoughby, Adrienne Harvey, and Kerr Graham, The Royal Children’s Hospital,
Melbourne, Australia.)

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Fig. 2-A

Fig. 2-B
Fig. 2-A Illustration depicting the diagnostic matrix of orthopaedic clinical decision-making for the management of ambulatory patients with CP, as
originally described by Davids et al.11. A detailed clinical history and physical examination are performed in the outpatient setting, with radiographic
evaluation of deformed skeletal segments. If multilevel surgery or generalized tone control is being considered, 3DGA is performed and a problem list can be
constructed. The final assessment of fixed musculoskeletal deformity is performed with the patient relaxed and under general anesthesia. (Images Courtesy
Gillette Children’s Specialty Healthcare, St. Paul, Minnesota.) Fig. 2-B Illustration depicting an example of the serial multidisciplinary treatment of spastic
diplegia over time in a facility with a gait-analysis service. A sample ambulatory patient with spastic diplegia is evaluated in the orthopaedic office as an
outpatient at between 4 and 7 years of age and is referred for multidisciplinary evaluation. The results of a formal evaluation (including physical examination
and 3DGA) are reviewed by multiple services. Spastic tone, birth history of prematurity, confirmatory neuroimaging findings, and good strength and
compliance lead to a decision for selective dorsal rhizotomy (SDR). Rhizotomy is performed, and inpatient rehabilitation begins thereafter. Reevaluation,
performed with 3DGA 1 to 2 years after selective dorsal rhizotomy, confirms resolution of spasticity and improvements in kinematics, gait indices, and energy
efficiency but demonstrates a remaining orthopaedic deformity. At the age of 10 years, SEMLS is planned to simultaneously correct all osseous and soft-
tissue deformities. Preoperative education is performed, medical and nutritional status is optimized, and SEMLS is performed. Follow-up with 3DGA at 1 to 2
years after SEMLS demonstrates the kinematic changes after the surgical correction of lever-arm dysfunction. Ortho 5 orthopaedic surgery, NSx 5
neurosurgery, PMR 5 physical medicine and rehabilitation, PT 5 physical therapy, PhD 5 gait-laboratory engineer, PE 5 physical examination, GDI 5 Gait
Deviation Index, and GPS 5 Gait Profile Score.

be reduced during non-weight-bearing
to achieve subtalar neutral, allowing
for the assessment of compensatory
foot motions during standing and
walking20,21.
Radiographic Assessment
The ambulatory child with CP should
have radiographic hip surveillance, with
pelvic radiographs being made at intervals
that reflect the increasing risk of spastic
hip subluxation with increasing GMFCS
levels22,23. Femoral neck-shaft angles are
also increased at higher GMFCS levels24.
Any child under consideration for lower-
extremity surgery should be evaluated

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Fig. 3
Data-collection tool for physical examination at the time of 3DGA for the patient described in the clinical scenario. All passive and
active motion, strength, reflexes, spasticity, and selectivity at each joint and muscular group are readily accessible, on a single page,
to the examiner and 3DGA team. A head-to-toe organization is used, with anatomical grouping according to musculoskeletal
segment (red boxes) and corresponding neurological examination (green boxes). DOB 5 date of birth, HS 5 hamstring, TF 5 thigh-
foot, BM 5 bimalleolar, MTP 5 metatarsophalangeal, and DF 5 dorsiflexion.

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TABLE I Useful Data Instruments for Evaluation of Ambulatory Patients with Cerebral Palsy and 3DGA*

Instrument Description

Patient questionnaires/classifications
GMFCS (Gross Motor Function Classification System)1,6 Ordinal 5-point system describing overall ambulatory ability in
children with CP, later expanded to 4 age groups: 2 to ,4, 4 to ,6, 6 to
,12, and 12 to 18 years
PODCI (Pediatric Outcomes Data Collection Instrument)26 Questionnaire with multiple dimensions (e.g., transfers, sport, mobility,
pain, happiness, global functioning, others). Designed to assess
change after orthopaedic intervention
FAQ (Gillette Functional Assessment Questionnaire)28 10-point patient-reported walking scale for all GMFCS levels
FMS (Functional Mobility Scale)29 Patient reported of functional mobility across 3 distances (5, 50, 500 m)
designed to represent home, school, and community ambulation
CP CHILD (Caregiver Priorities and Child Health Index of Life Measure of caregiver’s perception of HRQL of a child with CP
with Disabilities)30
Summaries of kinematic data
GDI (Gait Deviation Index)31 Multivariate score of overall gait pathology compared with typically
developing children
GPS (Gait Profile Score)32 and MAP (Movement Analysis The GPS is a score of overall gait quality comprising 9 gait variable
Profile)32 scores (GVSs). The MAP pictorially represents each of the 9 GVSs and
overall GPS
GGI (Gillette Gait Index)33 Composite of 16 kinematic parameters into a single gait score.
Previously termed “normalcy index”

*Data instruments are individual to the gait laboratory. The instruments listed above are commonly used but do not represent an exhaustive list.
HRQL 5 health-related quality of life.

with an anteroposterior pelvic radiograph
to assess femoral head migration percent-
ages, acetabular indices, and coxa valga.
Other radiographs are directed by
the deformity. Patients with overactive
quadriceps and crouch gait should be
evaluated with supine maximum-
extension lateral radiographs of the knees
to assess for patella alta and knee flexion
contracture. Planovalgus foot deformity
can be quantified with weight-bearing
anteroposterior and lateral radiographs of
the foot to document the talocalcaneal
angle, talus-first metatarsal angle, and
talonavicular coverage25. If 3DGA reveals
coronal plane malalignment, a scanogram
or equivalent is performed. With the
advent of low-dose, biplanar imaging
(EOS Imaging) and 3-dimensional re-
construction thereof, integrating imaging
with gait laboratory data opens new
means for correlating examination, radio-
graphs, and 3DGA.
3-Dimensional Gait Analysis (3DGA)
Instrumented gait analysis is available
at specialized centers. The reason for
referral to the gait laboratory must be
well documented. Specifically, patient
and family concerns about gait, func-
tion, and deformity should be
clarified26,27. Previous treatment,
including therapy, bracing, and tone
management, is reviewed. Family ques-
tionnaires, scores, and outcome instru-
ments may be obtained at the time of
analysis to quantify post-treatment
effects (Table I)1,6,26,28-33.
At our institution, 3DGA is used
for preoperative planning and for the
postoperative assessment of all ambula-
tory patients undergoing single-event
multilevel surgery (SEMLS). The main
components of 3DGA are a detailed
physical examination by an experienced
therapist working exclusively in the
motion laboratory, 2-dimensional video
from high-speed motion cameras, 3-
dimensional kinematics, kinetics, dy-
namic electromyography (EMG), and
normalized energy expenditure to elim-
inate the need to match for age, sex, and
stature. Variations in data-collection
methodology, processing, and output
exist between centers. For example, both
conventional and functional gait models
(2 methods by which joint centers and
rotational axes can be calculated) are
used at our laboratory. Also used are
multi-segment foot data, pedobarography,
muscle-length and velocity plots, gait-
stride parameters, summary scores of
overall kinematic deviation31, and pre-
dictive models of the outcome of certain
surgical procedures based on retrospective
in-house data34,35.
Kinematic data are presented as
graphic waveforms of joint or body-
segment motion in the coronal, sagittal,
and axial planes throughout the gait
cycle in relation to normal (Fig. 4-A).
Kinetic data detail internal joint mo-
ments on the basis of the relationship
between the ground-reaction force
vector and the joint center in question
as well as body-segment inertia and
mass (Fig. 4-B). Dynamic EMG mea-
sures electrical potential generation in
multiple muscle groups, identifying
pathological timing, spasticity, or
compensations (Fig. 4-C).

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Fig. 4
Figs. 4-A, 4-B, and 4-C Kinematic and electromyographic data for the patient described in the Clinical Scenario. Fig. 4-A Kinematic
gait data for transverse-plane motion at 4 skeletal or joint segments in the lower extremities (pelvis, hip, knee, and foot). Red 5 left
limb, green 5 right limb. The gait cycle is separated into stance (;65%) and swing (;35%) phases, the separation of which is
denoted by vertical lines. This patient has bilateral internal hip rotation and compensatory external pelvic rotation. Knee rotation is
borderline external, consistent with external tibial torsion. Foot progression angles are external, the left more external than right.
Fig. 4-B Sample kinetic data for sagittal plane motion. Near-normal internal hip and knee flexion-extension moments are achieved.
Normal stance phase involves 3 ankle rockers: first rocker begins with heel strike and continues until the foot is lowered to the
ground by eccentric tibialis anterior contraction, second rocker involves ankle dorsiflexion as the tibia and center of gravity progress
over the foot, and third rocker involves toe-off with concentric contraction of the triceps surae. The ankle kinetics reveal heel strike
on the left (dorsiflexion moment at initial contact through first rocker) but blunted first rocker on the right, consistent with
gastrocnemius-soleus tightness. Fig. 4-C Electromyographic data for several lower-extremity muscle groups. These tracings were
taken prior to selective dorsal rhizotomy. The shaded segments denote appropriate firing times during the gait cycle. There is
inappropriate swing-phase contraction of the rectus femoris but otherwise there is little inappropriate co-contraction of other
muscle groups.

3DGA provides a dynamic assess-
ment of ambulatory ability, joint motion,
and compensatory (or tertiary) motions
that are otherwise difficult to appreciate.
There are several common gait patterns
with distinct kinematic and kinetic pro-
files, for example, spastic hemiplegia36
and spastic diplegia37-39. Members of
the gait-analysis team convene to review
3DGA data. The team consists of the
experienced physiotherapist who col-
lected the data, a trained physician
interpreter (usually an orthopaedic
surgeon) who performs data analysis and
makes treatment recommendations, and
a biomedical engineer who provides in-
sight about the modeling or potential data
discrepancies40. At this meeting, all an-
tecedent historical, clinical, radiographic,
and gait data are discussed to formulate
treatment recommendations11.
Tone Management in Ambulatory
Patients with CP
A core principle in the management of
the hypertonic, ambulatory patient with
CP is tone management, categorized as
surgical or nonsurgical, focal or gener-
alized, and reversible or irreversible (Fig.
5). Pharmacological interventions in-
clude intrathecal or oral baclofen41 or
intramuscular botulinum toxin or phe-
nol42. Selective dorsal rhizotomy is a
neurosurgical procedure that perma-
nently reduces tone. Because it is irre-
versible, careful patient selection is
essential in order to achieve optimal
outcomes. The ideal candidate is a child
between the ages of 4 and 7 years with

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Fig. 5
Diagram depicting spasticity treatment. “Generalized” implies an effect over a large region of the body or the entire body, such as the
use of selective dorsal rhizotomy to reduce spasticity or the use of intrathecal baclofen (ITB) to reduce tone throughout the lower
extremities. Intrathecal baclofen or oral agents have diffuse systemic effects, which can be reversed upon discontinuation of use.
Selective dorsal rhizotomy (SDR) is an irreversible sectioning of afferent lumbosacral spine nerve rootlets, decreasing spasticity
throughout the lower limbs as a result. “Focal” therapies such as botulinum toxin (BTX) and phenol can be locally administered to
chemically weaken a target muscle, although the effects are reversible. Surgical lengthening of muscle or tendon units is semi-
reversible, often with eventual reconstitution of the lengthened segments with altered anatomical and functional properties.

GMFCS Level-I, II, or III ambulatory
status; birth history of prematurity; and
neuroimaging findings confirming peri-
ventricular leukomalacia9. Candidates
should exhibit sufficient strength and
motor control to maintain gait function
in the absence of spasticity, i.e., the pa-
tient should not be reliant on increased
tone for stability or propulsion (Table
II). Poor candidates are characterized
by mixed tone (i.e., more dystonia), less-
severe spasticity, established contrac-
tures (typically in older patients),
weakness, poor selective motor control,
or other diagnoses (particularly hereditary
spastic paraparesis). At our institution,
a multidisciplinary spasticity evaluation
is performed by an orthopaedist, neuro-
surgeon, and physiatrist with pediatric
expertise. 3DGA and assessment by a
physical therapist are routine. Medical
records, radiographs, and neuroimaging
studies are reviewed. This team-based
evaluation arrives at a consensus treat-
ment plan for the treatment of spasticity,
which might include selective dorsal
rhizotomy, intrathecal baclofen, other
pharmacotherapy, expectant manage-
ment, or orthopaedic surgery.
Selective dorsal rhizotomy is per-
formed according to the technique
described by Peacock, involving sec- lower-level motor skill with progression
tioning afferent spinal sensory rootlets to higher-level skills, selective motor
at the cauda equina or conus level; these control, and strengthening45. Rehabili-
rootlets are isolated by microdissection tation may include manual, treadmill,
and are selected on the basis of abnormal and robotic gait retraining46,47. Serious
responses to intraoperative electrical postoperative complications are rare,
stimulation43,44. At our institution, a and dysesthesia is expected and is treated
neurosurgeon performs the procedure aggressively with gabapentin. Selective
and a neurophysiologist and physiatrist dorsal rhizotomy has consistently re-
evaluate the intraoperative response to duced spasticity in several randomized,
stimulation. Generally, 20% to 40% of controlled investigations when com-
L1-S1 dorsal rootlets are cut, perma- pared with physical therapy alone48-51,
nently disrupting the abnormal reflex resulting in early improvements in
arcs contributing to lower-extremity walking speed, energy efficiency, and
spasticity. Physical therapy and a reha- gait scores45. In the study by Langerak
bilitation program are believed to be et al., 58% of 31 patients who under-
essential to the outcome, usually de- went selective dorsal rhizotomy had
manding inpatient focus on mastering improved GMFCS levels and had no
TABLE II Clinical Features of Patients Suitable for Selective
Dorsal Rhizotomy
Spasticity interfering with function (as opposed to dystonia)
Prematurity or neonatal insult/injury in late 2nd/early 3rd trimester
Periventricular leukomalacia (confirmed by neuroimaging)
Energy-inefficient gait
Fair or good strength/antigravity strength or better at hips and knees
Fair or good selective motor control at hips and knees
Good ability to cooperate with rehabilitation

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deterioration after an average of 21 years
of follow-up52. Despite documented
efficacy and safety, perceptions of a risk
of neurological complications, crouch
gait, and weakness have prevented
widespread acceptance of selective dorsal
rhizotomy53. The dependence on the
data gathered during 3DGA and the
multidisciplinary evaluation have led to
the broad application of this approach
at our institution.
Because of weakness induced by
and intense rehabilitation following se-
lective dorsal rhizotomy, this procedure
generally is not coupled with other sur-
gical procedures. Regardless of the
spasticity-management strategy, ortho-
paedic procedures will often be eventu-
ally recommended for the correction
of fixed osseous deformities54. Surgery
is delayed until late juvenile years
or adolescence if tone is successfully
managed42.
Single-Event Multilevel Surgery
The core principle of SEMLS is simul-
taneous correction of multilevel defor-
mity contributing to gait dysfunction in
an ambulatory patient with CP. The
procedures are performed under a single
anesthetic, hospitalization, and rehabil-
itation. An idealized paradigm involves
tone management and strengthening
until a child is 8 to 12 years old, at which
time all procedures are performed. As
muscles propel joints by force on an
osseous lever, surgery is aimed at the
treatment of fixed contracture and the
correction of lever-arm dysfunction9.
Construction of a Problem List
A problem list is constructed on the basis
of all available data, including data on
contracted muscles, joints, and osseous
deformities (this process is sometimes
referred to as the diagnostic matrix
concept11). Muscles and joints with
dynamic contracture secondary to hy-
pertonicity should be examined for fixed
contracture with the patient under an-
esthesia55. Underlying neurological
deficits are also carefully considered
when the treatment plan is being
devised.
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Preoperative Therapy Assessment and
Preoperative Education
At our institution, all patients scheduled
for SEMLS are offered preoperative ed-
ucation by a team of physical therapists.
The expected hospital course is
reviewed, including casting, pain man-
agement, therapy, transportation,
dressing, toileting, home equipment,
and postoperative coping. Many pre-
operative consultations can be made,
including therapeutic recreation, physi-
cal and occupational therapy, and die-
tetics. Some older patients who have
undergone SEMLS will have a planned
inpatient rehabilitation admission post-
operatively, depending on the proce-
dures and weight-bearing plan.
Muscle-Tendon Surgery
The clinical, radiographic, and kine-
matic indications for muscle-tendon
lengthening have been discussed in
detail11. For example, monoarticular
muscles (the iliacus, vasti, and soleus)
are often less affected in patients with
diplegic CP, are important for propul-
sion and stance-phase stability, and
generally are not lengthened. Biarticular
muscles (the psoas, hamstrings, rectus
femoris, and gastrocnemius) are prefer-
entially affected and are therefore con-
sidered for surgical lengthening. As a
general principle, fractional lengthening
or intramuscular recessions are used to
minimize weakness and to allow early
weight-bearing.
Psoas lengthening is used for
functionally limiting hip-flexion con-
tracture, decreased terminal-stance hip
extension, increased pelvic tilt, a
“double-bump” sagittal plane pelvic pat-
tern (a kinematic definition associated
with hip-flexor tightness), and increased
pelvic-tilt range of motion11,56. Medial
hamstring lengthening can be employed
for “short” or “slow” hamstrings (3DGA
definitions based on kinematics and
muscle-velocity plots) to improve exten-
sion in terminal swing, initial contact, or
loading response. Anterior pelvic tilt may
worsen if the hamstrings are over-
lengthened57 or may improve if the
hamstrings are severely shortened58. In
|
patients with a flexed-knee gait with
mild flexion contracture, semitendi-
nosus transfer to the adductor longus
improves knee extension at initial contact
and stance without affecting pelvic tilt59.
Rectus femoris transfer posterior to the
knee axis can be performed in patients
with stiff-knee gait to further improve
swing-phase knee flexion and to mitigate
cospasticity of the quadriceps and ham-
strings60. Gastrocnemius-soleus-
complex lengthening can improve pas-
sive ankle range of motion in patients
with persistent plantar flexion in stance
and swing phases and disruption of all
ankle rockers. This procedure can be
performed at 1 of 3 anatomical zones
according to the separation or conflu-
ence of the gastrocnemius-soleus fascia,
and the dose-response relationship of
lengthening at each level has been
quantified61. Zone-3 and often zone-2
lengthening should be avoided in pa-
tients with diplegia in order to prevent
weakness and iatrogenic crouch gait62.
Musculoskeletal modeling has shown
that a 1-cm lengthening of the soleus
results in a 50% decrement of force-
generation capacity62, and under-
lengthening is preferred if full correction
cannot be achieved. The soleus is par-
ticularly sensitive because of its bipen-
nate muscle-fiber orientation and short
fiber lengths. In cases of diplegia or
quadriplegia, the soleus rarely requires
lengthening. The gastrocnemius is
much less sensitive to lengthening and
typically is the only calf muscle length-
ening required to treat the ankle equinus
of diplegia.
Osseous Surgery
The importance of correction of lever-
arm dysfunction with osseous surgery is
now recognized. The composition of
SEMLS varies, but osseous procedures
have become increasingly utilized63.
Femoral anteversion can be identified by
means of examination19 and imaging64,
and internal hip rotation during gait is a
compensation to improve the abductor
lever arm that is compromised by inter-
nal femoral torsion65. Proximal femoral
derotation can be employed and mild
9
 | Te a m A p p r o a c h : S i n g l e - E v e n t M u l t i l e v e l S u r g e r y i n A m b u l a t o r y P a t i e n t s w i t h C e r e b r a l P a l s y
varus can be incorporated into the
osteotomy if hip dysplasia is present.
Crouch gait characterized by fixed knee
flexion, patella alta, and compensatory
overactive quadriceps can be treated
with distal femoral extension osteotomy
and patellar tendon advancement66 or
shortening55. Tibial malrotation can
be quantified by examination supple-
mented with computed tomography
(CT) or 3DGA. A normal bimalleolar
axis is 16° 6 6°67, and deviations can
cause tripping when torsion is internal or
diminished ankle push-off when torsion
is external. Supramalleolar tibial osteot-
omy is preferred because of the greater
risk of compartment syndrome with
proximal osteotomy68, and the need for
concomitant fibular osteotomy remains
controversial69. Foot reconstruction
to correct planovalgus foot alignment
may include lateral column calcaneal
lengthening70 to correct hindfoot valgus
and to improve midfoot instability,
combined with plantar flexion osteot-
omy of the first ray (metatarsal or medial
cuneiform) to correct forefoot supina-
tion. The derotations of external tibial
torsion and valgus foot deformity are
aimed at improvement of knee-extension
moment71, restoration of foot
stability, and correction of lever-arm
dysfunction through normalization of
the marked external foot progression9.
Careful assessment of gastrocnemius
length after foot correction often iden-
tifies contracture that was not apparent
preoperatively because of excessive
motion through the midfoot.
Clinical Outcomes After SEMLS
Historically, most surgical procedures
in ambulatory patients with CP were
muscle-lengthening procedures. Multi-
level soft-tissue releases, often involving
$4 or more muscular lengthenings per
limb without osseous surgery, were
reported to have good early kinematic
and functional results72. Some studies
of SEMLS performed in the 1980s
and 1990s reported 70% to 90%
of all procedures were soft-tissue
lengthenings73,74. These procedures
were successful for correcting deformity
10
but did not improve motor function,
with changes best appreciated in patients
who were marginal ambulators
(GMFCS Levels III and IV)75.
It was later observed that intra-
muscular tendon lengthenings miti-
gated hyperactivity and corrected
deformity but disrupted monoarticular
muscles62. This physical disruption,
coupled with underlying sarcomeric
elongation innate to skeletal muscle in
patients with CP, weakened antigravity
function and is undesirable for ambula-
tion and upright posture. Over-
lengthening or unnecessary lengthening
of the biarticular hamstrings worsens
anterior pelvic tilt16,59, and Achilles
tendon lengthening in patients with di-
plegia results in decreased ankle pushoff
(third rocker) and soleus weakness,
contributing to iatrogenic crouch
gait62,76. For patients with diplegia,
conservative calf lengthening is therefore
recommended. Long-term studies of
calf lengthening have demonstrated
sustained kinematic improvements and
low rates of calcaneal gait patterns77 but
a 20% to 35% rate of recurrence of
an equinus gait pattern75,78,79. Surgical
correction of lever-arm dysfunction has
demonstrated sustained correction of
femoral anteversion80-84 and safe cor-
rection of tibial torsion69,85. Recurrence
of femoral rotation can occur in patients
undergoing derotation before the age
of 10 years83,84,86, although recent evi-
dence suggests that the rate of prob-
lematic recurrence is #11%87. External
tibial torsion can recur regardless
of age, concurrent foot deformity, or
GMFCS88.
Conclusions about the effective-
ness of SEMLS treatment should be
deferred until 1 to 2 years postopera-
tively as decreased mobility occurs in
the first 6 months after surgery, followed
by improvement until 2 years
postoperatively72,75,89-93. A recent sys-
tematic review suggested that kinemat-
ics and kinetics, joint motion, gait
indices, and energy efficiency94 often
improve after SEMLS. Although the
quality of studies on the outcomes of
SEMLS improved from 1985 to 2010
according to MINORS (methodological
index for non-randomized studies) cri-
teria95, particularly with increasing
use of preoperative and postoperative
3DGA, most data are still Level-IV
evidence63. There is new evidence of
quality-of-life improvement after
SEMLS96.
Outcome tools assessing clinical
improvement after multilevel surgery
are varied. Although some retrospective
series have demonstrated an improve-
ment in GMFCS after SEMLS97-99, the
preoperative FMS (Functional Mobility
Scale) and GMFCS levels are generally
maintained at 1 to 2 years after sur-
gery100. We are aware of only 1 ran-
domized clinical trial investigating
SEMLS; that study demonstrated im-
provement in the GMFM (Gross Motor
Function Measure) and FMS and
maintenance of the preoperative
GMFCS level at 2 years postoperatively
compared with the findings after a
treatment of resistance training96. Pro-
spective analysis of these same patients
suggested that gait and functional im-
provements are maintained at 5 years
after SEMLS101 and that those with the
most abnormal preoperative gait pat-
terns garnered the most benefit102.
Rehabilitation and Follow-up
After SEMLS
There is little literature on the formal
rehabilitation after SEMLS63. At our
institution, a standardized regimen is
employed after most multilevel proce-
dures. Epidural anesthesia is utilized for
bilateral osseous surgery, and antispas-
modic medications (namely, benzodi-
azepines) are a mainstay of postoperative
pain control. An initial hospital stay of
4 to 5 days is followed by a period of home
stay, with the goal of improving comfort
and building family confidence. Con-
tinuous passive motion is often started
on the third day after certain procedures
(e.g., distal femoral extension osteotomy
with patellar tendon advancement or
rectus transfer) to mitigate joint stiff-
ness. Prone lying is employed to stretch
the hip flexors after femoral derotation
or psoas lengthening. Femoral, tibial,
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 Te a m A p p r o a c h : S i n g l e - E v e n t M u l t i l e v e l S u r g e r y i n A m b u l a t o r y P a t i e n t s w i t h C e r e b r a l P a l s y |

and foot reconstruction can all be pro-
tected with short-leg casting with a
derotational bar, which eases patient
transfers, and knee immobilizers can
ease spasm and encourage muscle
stretch. Range of motion and strength-
ening exercises and pivot transfers are
begun at 3 to 6 weeks, although many
surgeons will begin earlier weight-
bearing in patients with femoral osteot-
omies. At 6 weeks after SEMLS, most
casts are removed, orthotics are placed,
and therapy is advanced to improve
strengthening and mobility.
Inpatient rehabilitation for 1 to
4 weeks is considered if there has been
deterioration in ambulation, if maxi-
mum assistance is needed, if schoolwork
is suffering, or if the child is older or
heavier. The inpatient program focuses
on passive and active motion, strength-
ening, play therapy, mat and mobility
transfers, and ambulation with func-
tional or robotic gait retraining. When
a child has reached or surpassed the
preoperative functional status, he or she
is discharged to an outpatient program.
As discussed, postoperative 3DGA is
performed 1 year after SEMLS and prior
to implant removal as improvements in
gait and function are first quantifiable
at this time91,92.
The described approach involves
an idealized setting with ready avail-
ability of specialists, longstanding expe-
rience, and a clinical gait laboratory.
Socioeconomic factors, specialist avail-
ability, and access to 3DGA will neces-
sitate practice variations when caring
for an ambulatory child with CP.
Return to Clinical Scenario
Multidisciplinary evaluation of the pa-
tient was performed concomitant with
3DGA (Video 2). An anatomical prob-
lem list was constructed, including bi-
lateral femoral anteversion, excessive
internal hip rotation, bilateral external
tibial torsion and external foot progres-
sion angles, and bilateral gastrocnemius-
soleus tightness with adequate ankle
dorsiflexion in stance. SEMLS was rec-
ommended for simultaneous correction
of bilateral femoral and tibial lever-arm
dysfunction.
At the time of surgery, both limbs
were prepared and draped in a sterile
fashion with the patient in the prone
position. An assessment of true femoral
anteversion was performed with use of
a bone pin in the femoral neck axis. The
left and right femora were osteotomized
in the intertrochanteric region, dero-
tated 30° and 40°, respectively (final
femoral anteversion, 10°), and fixed with
blade plates, with maintenance of the
preoperative neck-shaft angle. With the
talonavicular joints in a corrected posi-
tion, the prone thigh-foot angle and
second-toe tests confirmed bilateral
excessive external tibial torsion of 20°,
concordant with swing-phase

Fig. 6
Figs. 6-A through 6-D The 10-year-old girl from the Clinical Scenario with spastic diplegia (GMFCS Level I) presented for post-rhizotomy evaluation
with excessive bilateral femoral anteversion and excessive medial hip rotation during gait. She underwent bilateral proximal femoral derotation osteotomy
in the intertrochanteric region. Figs. 6-A and 6-B Preoperative (Fig. 6-A) and postoperative (Fig. 6-B) radiographs demonstrating maintenance of
neck-shaft angle and osseous union at 1 year after derotation of 30° (left femur) and 40° (right femur). Fig. 6-C Because of concomitant external foot
progression and external tibial torsion, internal-rotating osteotomies of both tibiae were performed. Fig. 6-D Graphical representation of normalized
oxygen consumption (y axis) and dimensionless walking speed (x axis) at the time of 3DGA. The numbering of data-collection time points proceeds
from oldest (4) to most recent (black dot). After Time Point 3, oxygen consumption was 302% of normal and the decision was made to perform selective
dorsal rhizotomy. This procedure resulted in improvement in normalized oxygen consumption and maintenance of normal walking speed (Time Point 2).
After SEMLS, normalized oxygen consumption was 162% of normal and walking speed was maintained (black dot 5 current).

AUGUST 2017 · VOLUME 5, ISSUE 8 · e10 11

 | Te a m A p p r o a c h : S i n g l e - E v e n t M u l t i l e v e l S u r g e r y i n A m b u l a t o r y P a t i e n t s w i t h C e r e b r a l P a l s y
kinematics. Internal tibial derotation
was then performed through the supra-
malleolar region, followed by fixation
with small-fragment plates and reas-
sessment of correction on the basis of the
thigh-foot angle and the second-toe test
(Figs. 6-A, 6-B, and 6-C). After tibial
derotation, Silfverskiöld testing revealed
ankle dorsiflexion of 10° and 15°, ap-
propriate for maintaining second rocker.
Instrumented gait analysis at 1 year
(Video 3) demonstrated improved di-
mensionless walking speed, energy ex-
penditure (Fig. 6-D), and FMS level,
with maintenance of function at
GMFCS Level I.
Andrew G. Georgiadis, MD1,
Michael H. Schwartz, PhD1,
Kathryn Walt, PT1,
Marcia E. Ward, MD1,
Peter D. Kim, MD1,
Tom F. Novacheck, MD1
1Departments of Orthopaedic Surgery
(A.G.G., T.F.N.), Physical Therapy (K.W.),
Physical Medicine and Rehabilitation
(M.E.W.), and Neurosurgery (P.D.K.),
and the James R. Gage Center for Gait
and Motion Analysis (T.F.N, M.H.S.),
Gillette Children’s Specialty Healthcare
St. Paul, Minnesota
E-mail address for A.G. Georgiadis:
Andrew.g.georgiadis@gmail.com
ORCID iD for A.G. Georgiadis: 0000-0002-
3624-8273
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