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article info
abstract
Keywords:
Successful treatment of patients with neonatal brachial plexus palsy (NBPP) begins with a
Brachial plexus
thorough understanding of the anatomy of the brachial plexus and of the pathophysiology
Obstetric
of nerve injury via which the brachial plexus nerves stretched in the perinatal period
Neonatal
manifest as a weak or paralyzed upper extremity in the newborn. NBPP can be classied by
Surgery
systems that can guide the prognosis and the management as these systems are based on
Rehabilitation
the extent and severity of nerve injury, anatomy of nerve injury, and clinical presentation.
Outcomes
Introduction
The management of patients with neonatal brachial plexus
palsy (NBPP) begins with the understanding that stretching the
nerves of the brachial plexus in the perinatal period manifests
as a weak or paralyzed upper extremity, with the passive range
of motion greater than the active, in a newborn.
Classication
The most useful classication scheme for the management
and the prognosis of NBPP was proposed by Gilbert and
E-mail address: ljsyang@med.umich.edu
http://dx.doi.org/10.1053/j.semperi.2014.04.009
0146-0005/& 2014 Elsevier Inc. All rights reserved.
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Group
I
II
III
IV
C5 and C6
C5, C6, and C7
C5, C6, C7, C8, and T1
C5, C6, C7, C8, and T1 with
Horner's syndrome
Rate of full
spontaneous recovery
(%)
!90
!65
o50
!0
C6, C7, C8, and T1). Group IV manifests as a ail arm with the
additional presence of Horner's syndrome (ptosis, meiosis, and
anhydrosis) of the ipsilateral eye and face, presuming injury to
all the nerve roots of the brachial plexus with a very proximal
injury to the lower nerve roots. When this classication system
is used between 2 and 4 weeks after birth, it facilitates
determination of the extent of injury to guide prognosis and
subsequent management.
Other classication schemes that guide the prognosis and the
management rely upon the anatomy and physiology of the
nerve injury. Sunderland reported a physiologic scheme comprising ve types of pathology in increasing severity: (1)
neurapraxia (transient nerve injury that may result from a brief
ischemic episode or from any form of compression, demyelination, or axonal constriction or stretch); (2) axonotmesis
(transient or permanent nerve injury in which the majority of
the supporting structures of the nerve, endoneurium, perineurium, and epineurium are preserved, but disruption of the
axonal nerve bers is present); (3) lesion of the axon and the
endoneurium (likely resulting in permanent nerve injury); (4)
lesion of the axon, endoneurium, and perineurium (likely
resulting in permanent nerve injury); and (5) complete transection of the entire nerve (permanent nerve injury).5 For
example, most nerve reconstruction surgeons manage patients
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Assessment scales
Assessment of motor function
Assessment scales in NBPP are used to gauge the extent of
injury, prognosticate potential recovery, and determine treatment. Commonly used scales primarily focus on joint angles
or muscle activation. Muscle power is generally expressed via
the U.K. Medical Research Council scale for muscle movement (MRC scale) (Table 2). This scale provides structured
grading of individual muscle groups, but it does not provide
any information about the overall function of the limb or the
child. Because the MRC scale requires voluntary cooperation,
it is difcult to apply in newborns but can be inferred from
Table 2 The UK Medical Research Council scale for
muscle movement (MRC scale) for muscle power.
M0No detectable muscle contraction
M1Palpable muscle contraction without movement
M2Movement in a horizontal plane (gravity eliminated)
M3Movement overcoming the pull of gravity
M4Movement overcoming resistance beyond the pull of gravity
M5Normal strength
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Muscle grade
Gravity eliminated
No contraction
Contraction, no motion
Motion r1/2 range
Motion 41/2 range
Full motion
0
1
2
3
4
Against gravity
Motion r1/2 range
Motion 41/2 range
Full motion
5
6
7
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Shoulder function
Stage
Flail shoulder
Abduction or exion to 451; no active external
Abduction o901; external rotation to neutral
Abduction 901; weak external rotation
Abduction o1201; incomplete external rotation
Abduction 41201; active external rotation
Normal
0
I
II
III
IV
V
VI
Score
Flexion
Nil or some contraction
Incomplete exion
Complete exion
1
2
3
Extension
No extension
Weak extension
Good extension
0
1
2
Extension decit
01301
301501
4501
225
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0
#1
#2
Description
Grade
0
I
II
III
IV
Supplementary studies
Electrodiagnostic examination
Supplementing the physical examination with electrodiagnostic (EDX) ndings is helpful to determine whether spontaneous recovery is occurring or whether nerve repair/
reconstruction will be benecial, and a thorough discussion
of EDX in NBPP has been published.37 Early referral of neonates with NBPP and extensive nerve injury may improve
outcomes; e.g., the early presence or absence of elbow
extension or elbow exion on clinical examination and of
motor unit potentials on electrodiagnostic examination in the
biceps muscle correctly predicted whether lesions were mild
or severe with respect to long-term involvement in 8594% of
infants.38
EDX ndings can provide information regarding the location, severity, and extent of NBPP. For example, identication
of an avulsed nerve root is critical to NBPP management;
avulsion injuries are considered neurotmetic, spontaneous
recovery does not occur, and surgical nerve reconstruction is
recommended early. Nerve root avulsions are preganglionic
injuries (the motor cell body is detached from its axon, but
the sensory cell body is continuous with its distal axon, Fig. 2)
and generally do not lend themselves to nerve graft repair but
are amenable to nerve transfers if appropriate donors exist.
In contrast, ruptured (Fig. 3) nerve roots/trunks (postganglionic neurotmetic or rarely axonotmetic injuries; EDX studies can identify but cannot distinguish axonotmesis from
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Electromyography (EMG)
EMG comprises assessment of the muscle at rest and then
during voluntary movement. At rest, the muscle is evaluated
for signs of abnormal spontaneous activity, which is consistent with lack of nervous input. The presence of brillation
potentials and positive sharp waves indicates that nerve
degeneration is occurring. MUAPs are evaluated by assessing
the amplitude, phase, duration, and ring rate-related to
force. Theoretical and animal studies suggest that neonates
likely develop denervation potentials earlier than the adult
time period of 1421 days.41,42 The quality and the quantity of
MUAPs in infants and children are also different from the
standard denition with adult norms. A normal adult MUAP
is triphasic, infant MUAPs are often biphasic, and the amplitude of MUAPs in children aged 03 years ranges between 200
and 700 V.43,44 The recruitment pattern is difcult to elicit as
voluntary activity is not easily controlled or graded since
infants cannot follow commands.
When assessing voluntary muscle activation, EDX focuses
on the presence and the number of voluntary motor units
present and characterizes their morphology recruitment
patterns. Nerve regeneration and reinnervation of muscles
is indicated by collateral sprouting from surviving axons,
appearing as polyphasia, large amplitude units, or increased
duration potentials. Evidence of axonal regeneration is suggested by the presence of nascent units, which are small in
amplitude, highly polyphasic, and have prolonged duration.
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Radiologic examination
In the immediate postnatal period, radiographic imaging may
be indicated urgently to assess for the presence of clavicular
or humeral fractures or diaphragmatic asymmetry, but the
focus of this section will be imaging of the nervous brachial
plexus elements. Keep in mind that neurological decits in
NBPP may mask symptoms due to coincidental, but more
distal, nerve lesions resulting from fractures of the long
bones in the arm,50 and ignoring these coincidental lesions
can lead to decreased outcomes after intervention.
Traditionally, imaging studies of the brachial plexus in NBPP
are performed only when microsurgical nerve repair/reconstruction is indicated due to the potential complications from
the imaging procedure. However, with modern magnetic resonance imaging (MRI) techniques, imaging may be taking its
place in the management arena. Like EDX, radiologic investigations supplement the clinical presentation by attempting to
provide information on the type, location, and extent of nerve
injury. Ideally, radiographic studies of the brachial plexus
should delineate the course of the pathoanatomy of the cervical
spinal roots, from their origin as dorsal and ventral rootlets at
the spinal cord through the vertebral foramina, the extraforaminal spinal nerve roots, the trunks, the divisions, and the
cords of the brachial plexus down to the terminal branches
innervating the muscles of the arm. At present, this remains an
unrealistic ideal as does the functional imaging of central
connections of the brachial plexus in babies. However, the
reported sensitivity of computerized tomography/myelography
(CTM) for detecting a postganglionic rupture was 58.3% and
72.2% for preganglionic nerve root avulsion.49 Consequently,
radiographic imaging is concentrated on these injury types, but
more specically on nerve root avulsions.
Later, a child fails to recover as predicted or new neurological symptoms develop, radiographic examination can
reveal arachnoid cysts compressing the spinal cord or herniation of the spinal cord into a large pseudomeningocele51
and supercial siderosis.52
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Ultrasonography
Few, if any, reports exist regarding the use of ultrasonography (US) in NBPP. We have had some experience applying US
preoperatively and comparing the preoperative CTM or MRI
and intraoperative pathology with the US results. In our
experience, the utility of US is complementary to that of
CTM or MRI as US can give information on the muscles and
the location of the brachial plexus neuroma to facilitate the
formation of strategies for nerve reconstruction. Furthermore, to avoid radiation from x-rays, US can be used to
assess for diaphragmatic movement (phrenic nerve function).
Computed tomography/myelography
CTM has been the long-preferred NBPP diagnostic tool at
most specialty brachial plexus centers. Many investigators
reported high sensitivity in the assessment of intradural root
avulsions,5355 and it permits separate evaluation of the
ventral and the dorsal nerve roots in the intradural space
(Fig. 4). However, the disadvantages of CT-myelography are
the need for general anesthesia and lumbar puncture for
intrathecal contrast introduction, as well as radiation exposure. Other difculties include the inability to determine the
correct spinal level,56 but the absence of hypodense root
shadows with or without a pseudomeningocele is suggestive
for nerve root avulsion/preganglionic injury. Note that the
presence of a pseudomeningocele is not an absolute proof of
root avulsion. CT-myelography cannot assess for ruptures
(postganglionic injury) or other types of lesions of nerve roots
within the foramen or in their extraforaminal course.
Treatment
Conservative management/rehabilitation and therapy
Plain radiographs
X-ray examinations can show fractures of the cervical spine,
humerus, or clavicle in the newborn. A chest x-ray can assess
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root avulsions with no spontaneous recovery. Correspondingly, a signicant percentage of NBPP patients do not regain
full arm function, so the principles of rehabilitation remain
constant: maintain range of motion (ROM) at all relevant
joints to avoid contracture formation, encourage muscle
strengthening, prevent compensatory movement patterns,
and, most importantly, promote normal childhood
development.
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Infants
Motor training should begin as early as possible to stimulate
activity in denervated muscles, to enable muscles to be
activated as soon as nerve regeneration has taken place, to
prevent or minimize soft tissue contractures, and to minimize ineffective substitution movements. Motor training
should continue for as long as nerve recovery is still occurring
(potentially for years).
In addition to range-of-motion home exercises, parents
should be educated regarding the need for tummy time at
each diaper change to promote symmetrical head rotation
and positioning. Torticollis is an abnormal head posture,
including ipsilateral tilt, contralateral rotation, and translation, and has been associated with NBPP.61 Persistent
torticollis can lead to plagiocephaly and facial asymmetry;
deformational plagiocephaly can be appreciated as early as 6
weeks of age with a preexisting diagnosis of torticollis.62 For
infants with torticollis, parents should be encouraged to vary
the position of the infant's head during play, feeding, and
sleeping. Use of positioning wedges may be helpful. Home
programs using neck stretches to address tightness of the
sternocleidomastoid muscle may be required for some
infants and should be taught to families by appropriately
trained therapists, and aggressive intervention is rarely
required.
In some instances, a newborn will require a hand/elbow
splint prior to discharge from the hospital (e.g., tightness of
the nger joints and/or signicant atrophy of the thenar
eminence, especially when associated with Horner's syndrome). An elbow exion splint may be indicated if subluxation is present. Extreme hyperextension of the elbow reects
absent biceps muscle activity in the context of intact triceps
muscle activity, causing severe muscle imbalance. Passive
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range-of-motion exercises for elbow exion should be performed with careful attention to position of the forearm in
supination or pronation (whichever position prevents subluxation from occurring).
Pain should be absent during newborn range-of-motion
exercises. If pain is present, re-evaluate for skeletal injury.
Sensory alterations can be present and manifest as the absence
of or impaired sensation in all or part of the extremity, based
upon the pathoanatomy of the nerve injury. With altered
sensation, hyperesthesia and allodynia are expressed in the
newborn with fussiness or chewing of the affected part of the
arm. Desensitization can relieve the symptoms and can be
achieved by the use of a rm touch versus a light touch, the use
of infant massage, a variety of texture inputs from fabrics, or
vibratory input from infant toys.
If a skeletal fracture is present, the arm should be immobilized using a sling, with the shoulder adducted and internally rotated and the elbow exed at 901 so that the arm rests
upon the infant's chest for the rst few weeks. The newborn
should be lifted by scooping the newborn under the buttocks
with one hand and under the head with the other versus
lifting the infant under the axillae. Teaching families to dress
the involved extremity rst and undress it last can reduce
unnecessary movement of the involved extremity during the
healing phase of the fractured area(s).
Once the infant's muscles are stretched and prepared for
activity, elicitation of active (versus passive) range-of-motion
exercises can be encouraged by stroking, tapping, or vibrating
the muscle belly. Elicitation can occur in gravity-eliminated
positions, progressing to antigravity positions, and ultimately
in weight-bearing positions that are developmentally appropriate for the patient. Vibration/stroking can be used to elicit
biceps contraction or elbow exion to achieve movement
patterns of the hand to the face or the mouth, elbow
extension such as batting at toys overhead, and wrist extension patterns to facilitate reaching for toys. The therapy
sessions should include interventions that facilitate the
patient's current level of generalized development. The
impact of the weak arm upon developmental milestones
should be a major focus of every therapy session.
Infants with NBPP learn quickly to adapt to their development with a unilateral bias since there is generally no
accompanying cognitive decit. For example, to preclude
the bias, progression toward symmetrical development
begins with learning to roll to both the right and the left
sides. Some infants master the commando crawl, while
others will not learn to crawl and will progress directly from
sitting to walking. Protective reactions in the affected extremity are often delayed or weak, yet they must be a focus of
therapy. A small therapy ball can be used to develop forward
protective reactions in the prone and the sitting positions.
Similarly, with the increasing popularity of the back-tosleep campaign, prone activities and bilateral neck rotation
must be encouraged to promote maximal function of the
recovering muscles and to prevent plagiocephaly. Use of
inhibitory or facilitative Kinesio-taping (KMS, LLC, Albuquerque, NM) and dynamic weight-shifting activities can maximize the development of proximal stability within the trunk
and the shoulder area. Flexibility throughout the neck and
the trunk is imperative for optimal shoulder range of motion.
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Pain management
Pain in NBPP, if it occurs, usually does so as the infant
matures, but its presence is difcult to detect in infants and
in young children. In those with chronic disablement, discomfort results from overuse movements, such as keyboarding or performing a task at home or at school. Treatment
goals for the child with pain include the following: (1)
reducing the pain with oral or cutaneous medications, (2)
determining the substituted movement patterns that are
causing the pain, and then (3) teaching the patient to move
more effectively (e.g., use of adaptive equipment)in such a
way that minimizes pain as well as overuse of the adjacent
joints during that particular task.
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Nerve reconstruction
The appropriate selection of NBPP patients who may benet
from surgical intervention remains controversial, and several
different paradigms have been reported.6668 Most NBPP
surgeons agree that all patients with neurotmetic lesions or
nerve root avulsions are reasonable surgical candidates.
Some consider absent or signicantly impaired hand function, in the context of a ail arm at birth, to be an absolute
indication for nerve surgery as soon as the infant reaches the
age of 3 months69 and/or by 34 months of age for those
patients who demonstrate no spontaneous recovery of
shoulder external rotation and elbow exion/forearm supination at that time. Some surgeons proceed with surgical
exploration if the true shoulder and elbow movement is
absent by 6 months of age, since they feel that the potential
benets from repairing neurotmetic lesions generally outweigh the risks of negative exploration.70 Surgery for NBPP is
rarely performed before 3 months of age and is almost always
performed before 9 months of age.
Early assessment by a specialty center allows institution of
conservative management options, determination of the
severity of the brachial plexus lesion(s), addressing of social
and psychosocial issues, and appropriate time needed to
consider the recommended treatment options for the
parents/caretakers.
Post-operative care
After nerve repair/reconstruction, some surgeons place the
infant's upper body in a prefabricated cast to limit movement
of the head and the affected arm for 2 weeks, whereas other
surgeons do not immobilize. Patients undergo clinical examinations at our outpatient clinic initially at frequent intervals
and then at 6-month intervals. Recovery of function can
occur up to 45 years after nerve reconstruction.
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Outcomes
Natural history
The natural history of NBPP, around which the management
and the prognosis revolve, remains the subject of speculation
and controversy in the literature. The complete potential
scope of NBPP is difcult to dene because of the variety of
theoretical combinations of lesions within the elements of
the brachial plexus; e.g., because the brachial plexus is
comprised of ve roots, three trunks, six divisions, three
cords, and ve terminal branches, thousands of theoretically
different brachial plexus lesions are possible for the nerves
alone, even without regard to additional musculoskeletal
issues, although the most common form of NBPP is the
supraclavicular upper trunk lesion.
Further difculties with the sheer determination of the
natural history include the denition of recovery and the
potential bias introduced by the referral patterns of reporting
physicians71,72 since many patients with Erb's palsy recover
spontaneously and are not referred to the specialists who
publish most reports. With these caveats in mind, some
authors provide an encouraging view of the natural history
of NBPP with over 80% occurrence of a favorable functional
outcome or complete recovery,7378 whereas other authors
provide a opposing view, with less than 50% with good
recovery or freedom from persisting disabilities.72,7983
As the absence of spontaneous clinical improvement persists over increasing time, the potential for recovery diminishes,74,84,85 and early recovery (clinical improvement within
weeks with functional recovery by 34 months) is generally
associated with favorable outcomes.74,86 The predictors of
recovery described above use simple clinical muscle assessments (e.g., Narakas Classication). Other authors have constructed paradigms based on more complicated statistical
analyses of multiple independent clinical variables.38,75,87
Regardless, a number of children appear normal and seem
to have recovered function, but the affected extremity is not
equally functional, when measured by more appropriate
sensitive tests.
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Surgical outcomes
Primary nerve reconstruction
The indications for surgical nerve reconstruction in NBPP
vary among different practitioners, with the exception of
Narakas Group III and IV lesions, for which nerve reconstruction is generally recommended. For example, some feel that
the inability to pass the cookie test at 9 months is a
reasonable indication for surgery,88,89 whereas others rely
upon the towel test (inability to remove a towel covering
the faces at 6 months with the affected arm)90 or the lack of
biceps function at 3 months of age.91 Many practitioners use a
combination of these clinical observations supplemented by
ancillary studies to guide their practice, standard guidelines
or critical pathways have been developed.
Similarly, many challenges (including the variability of
anatomical lesions in the complex brachial plexus structure
and adjacent musculoskeletal elements, differing surgical
Conclusion
In the 21st century, infants who sustain NBPP have an overall
optimistic prognosis, with the majority recovering adequate
functional use of the affected arm. However, of utmost
importance are (i) early referral to interdisciplinary specialty
clinics that can provide up-to-date advances in clinical care
and (ii) increasing research/awareness of the psychosocial
and patient-reported quality-of-life issues that surround the
chronic disablement of NBPP.
refere nces
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88.
89.
90.
91.
92.
93.
94.
95.
96.
97.
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98.
99.
100.
101.
102.
103.
104.