Download Restoring function for children with brachial plexus birth palsy

Restoring function for children with
brachial plexus b
T
By Melissa Miller, MSN, RN; Allison Allgier, OTR/L; and Emily Louden, MPH
The brachial plexus is a complex system of nerves
exiting the spinal cord at the C5-T1 levels. These
nerve roots then branch out to support motor and
sensory function for the upper extremities (see The
brachial plexus). William Smellie was the first to
record a brachial plexus injury associated with birth,
and his findings were published in 1768. In 1872,
Guillaume Duchenne, a French physician, coined
the term, ‘obstetrical brachial plexus palsy’ and clearly
18 OR Nurse 2013 March
stated the etiology of the injury. He was then
followed in 1874 by Wilhelm Erb, a German
neurologist, who outlined the anatomy associated
with brachial plexus injuries.1
Incidence
Brachial plexus birth palsy occurs in 1.5 per thousand live births and is thought to be related to the
birthing process.2 Known risk factors for brachial
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birth palsy
plexus birth palsy include large birth weight, maternal
diabetes, shoulder dystocia, prolonged labor, instrumented delivery, and breech presentation. Despite
these, less than half of infants with brachial plexus
palsy have known risk factors.2 The injury is identified immediately after birth, and the degree of injury
for each nerve found in the brachial plexus can range
from neurapraxia to avulsion (see Types of injuries).
Evaluation
Referral to a specialty center is recommended, as
serial physical exams are the best method for
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evaluation. During the assessment, a number of
methods may be used to document a patient’s
level of function, including the Toronto Score,
Mallet Classification, Active Movement Scale (AMS),
and the Narakas Classification. The Toronto Score
is used to quantify upper extremity function using a
0 to 10 scoring method and was designed to predict
outcomes in patients with brachial plexus birth palsy
at 3 months of age.3 The AMS is comprehensively
used to document muscle group function controlled
by the entire brachial plexus; the reliability of these
instruments has been documented.3
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Restoring function for children with brachial plexus birth palsy
Narakas developed his classification in an
attempt to categorize brachial plexus injuries by level
of involvement. Group I refers to C5 to C6 involvement, Group II designates C5 to C7 involvement,
Group III indicates a total plexus injury, Group IV
signifies the most severe form, characterized by a flail
extremity with a Horner syndrome (see Child with a
total brachial plexus birth palsy).4 Horner syndrome
results from avulsion of T1 and is signified by ptosis,
miosis, and anhidrosis on the affected side.5
Spontaneous recovery of function in the upper
extremity has been reported in 64% of Narakas I and
II patients. These patients demonstrated biceps muscle
recovery at 3 months of age, while only 9% of patients
in the Narakas III and IV group had the same result.6
Nerves regenerate at a rate of 1 mm/day or 1 in.
(2.54 cm)/month. It’s common for the shoulder to
begin functioning before the elbow. Through observation, the best indication of a good outcome is how
quickly the biceps muscle begins to function against
gravity. The assessment of biceps function is best
isolated when the patient is placed on the involved
side with the arm straight out at shoulder height.
Clear biceps function against gravity and can be
palpated and seen when the elbow bends during
the hand-to-mouth motion.
The brachial plexus
Preoperative diagnostic testing
Diagnostic testing is recommended preoperatively
to identify concurrent injuries. Humerus or clavicle
fractures may be identified with X-rays. Shoulder
dislocation, which results from altered muscle function and growth, may be ruled out with a shoulder
ultrasound. An electromyogram (EMG) and/or
magnetic resonance imaging (MRI) of the brachial
plexus may be recommended to gain further
information regarding the actual lesions and
muscle function impacted.
Nonsurgical intervention
Individualized treatment plans are developed
following the initial assessment and often include
occupational or physical therapy to optimize each
patient’s function. Therapy may begin as early as 1
to 2 weeks of age and usually occurs once a week,
so family education and home exercise programs
are important aspects of treatment. Therapy may
include aquatics, constraint-induced movement therapy, orthoses, therapeutic taping, and neuromuscular
electrical stimulation.7 Appropriate intervention is
important to maximize motor function, promote
increased signals to the brain, and minimize
development of deformity. In addition to therapy, a
nonsurgical option may include the use of onabotulinumtoxinA to improve muscle balance around the
shoulder and/or elbow.
C5: Moves the shoulder
C6: Flexes the elbow and wrist
C7: Sensory trunk, which straightens the elbow
and allows wrist movement
C8: Flexes wrist and fingers, extends the fingers,
and provides hand movement
T1: Controls hand muscles
Types of injuries
• Avulsion: The nerve is pulled away from the
spinal cord
• Rupture (Neurotmesis): The nerve is torn but
still attached to the spinal cord
• Neuroma: The nerve is surrounded by scar
tissue
• Neurapraxia: The nerve has been stretched
Image provided as a courtesy of the authors and Cincinnati
Children’s Hospital Medical Center.
20 OR Nurse 2013 March
Image provided as a courtesy of the authors and Cincinnati
Children’s Hospital Medical Center.
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Surgical intervention
Children who don’t have a spontaneous recovery
within the first few months of life are typically recommended for microsurgery to restore function to
the affected upper extremity. Each case is individualized based on the location and extent of the injury.
The severity of the injury is determined preoperatively
by the degree of functional return in the shoulder,
biceps, and hand. The timing of surgery is debatable
amongst multiple authors. Some authors recommend
intervening as early as 3 months of age, while others
recommend intervening beyond 3 to 6 months of
age.5,8-12 The general consensus is that those infants
who clinically appear to have global lesions (Narakas
III and IV) and the presence of Horner syndrome are
considered for surgery by 3 months of age.8-10 Early
intervention in global lesions is important to minimize the loss of motor function and maximize the
recovery time.8
The actual procedure entails exploring the brachial
plexus and then restoring function with nerve grafts
and/or nerve transfers. During the exploration, the
plexus is examined for the presence of a neuroma,
injury severity, number of nerve roots involved, and
motor response distal to the injury. The information
gathered from the exploration determines whether
nerve grafting, nerve transfers, or a combination of
both are necessary.9,12
Preoperative assessment
In addition to determining the rate and degree of
return of function in the affected extremity, the
infant needs to be evaluated for potential anesthesia
and surgery risks. The infant is screened for a history
of cardiac and respiratory disorders, family history of
blood disorders, malignant hyperthermia, and pseudocholinesterase deficiency. Parents are questioned
about the family’s previous exposure to anesthesia
and any related complications. A preoperative headto-toe assessment performed by the primary care
provider is reviewed for any risk factors, such as
underlying respiratory disorder, upper airway congestion, fever, and skin rashes. A complete listing of
known allergies and medications, in addition to
N.P.O. status for solids and liquids, is collected
preoperatively.
A few important aspects to consider when performing a preoperative assessment are the symmetry
of the chest during respirations and the baseline
functional evaluations utilizing the Toronto Score
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Child with a total brachial plexus
birth palsy
Photo provided as a courtesy of the authors. Permission to use was
obtained at the time the photo was taken.
and AMS. Chest ultrasounds are performed preoperatively to evaluate the function of the diaphragm
and to determine if the phrenic nerve was injured.
The phrenic nerve arises from C3 to C5, and a
weakening in the diaphragm reflects a possible
upper trunk or global injury.8,12 The preoperative
evaluation of the diaphragm is used to determine if
intercostal nerves could be transferred (if necessary)
and is later compared with the postoperative evaluation. The serial evaluations of function can determine
if there have been improvements in the overall
function. The clinical picture determines if the child
is a surgical candidate and predicts which nerve roots
are affected.
Preparing for surgery
Patient education is an important aspect of preoperative care. Many families may have some mistrust in
the healthcare system because of the belief that the
injury could have been avoided. Listening to the
family’s story and providing patient education about
the injury and treatment is crucial in instilling trust.
The family should be informed of the different severities of injury, clinical findings, and treatment options.
In addition, detailed information about the surgical
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Restoring function for children with brachial plexus birth palsy
procedure and the recovery
can be stored at this temperatime should be discussed with
ture for 48 hours.13 The nerve
the families.
and muscle stimulator is utilized
The OR and same-day surgery
to determine the quality of the
nurse should be knowledgeable
motor response of the nerve
of the policies concerning the
and can identify nerves, such as
presence of a parent/guardian
the phrenic, spinal accessory,
during induction of the child.
and others that may be transThe induction procedures are
ferred. The microscope should
explained by the anesthesia staff
be prepped, draped, and tested
to the parents in the preoperafor proper functioning, and
tive phase. The families are prothe lamp should be set at
The nerve and muscle
vided with frequent updates
the lowest feasible levels to
stimulator is utilized to
throughout the case, since most
prevent burns.
determine the quality of
brachial plexus nerve repairs are
Proper positioning of the
the motor response of
4 to 8 hours in length.
patient prior to applying the
the nerve.
A preoperative briefing with
sterile drapes is very important
the surgeons, anesthesia staff,
and should be discussed with
and OR nurses should take place prior to the case,
the surgeon prior to the case. Most surgeons prefer to
so the entire team is aware of the plan of care. The
have the patient situated in a supine position if an
infant’s medical history, proper positioning, adminisexploration is going to take place. The infant is
tration of neuromuscular blocking agents, prepping
placed on the operating table, and the neck is
of the extremities, and the necessary equipment
extended with the head turned to the opposite side
should be discussed. Many times during brachial
of where the incision will be made. Sterile towels or
plexus repairs that involve nerve grafting, three of
a shoulder roll is placed between the shoulder blades
the four extremities need to be prepped, so deterto promote extension of the neck and to open the
mining which extremity to place the BP cuff and
supraclavicular region. All pressure points should be
insert the I.V. line is important to maintain sterility.
meticulously padded, including the head. If the surgeon is confident that nerve grafting will take place,
Also, discussing which side of the mouth to secure
the patient may be placed in a prone position to
the endotracheal tube based on which side of the
neck the surgeon will be making the incision is
harvest the sural nerve from the back of the legs and
important to prevent accidental extubation of the
then turned to a supine position.12 If nerve transfers
patient during the case. Neuromuscular blocking
are indicated, the patient is placed on a bean bag
and positioned in a lateral decubitus position to
agents are not administered during brachial plexus
allow exposure of the posterior aspect of the affected
repairs–so the surgeons can internally stimulate the
shoulder. The affected upper extremity and bilateral
nerves of the plexus–and evaluate the response.
lower extremities are prepped and draped free. A
Discussing these plans prior to the case starting
urinary drainage catheter is placed to monitor urine
will reduce errors, and clarify what is needed and
output and maintain sterility of the lower extremiexpected from the surgeons.
ties. Once the infant is positioned and secured to the
OR table, a forced-air warming device is placed over
Intraoperative set-up
the patient to control body temperature.
Set-up of a brachial plexus repair case entails proper
patient positioning, verifying that the microscope is
Brachial plexus exploration
available, a nerve and muscle stimulator is present,
Brachial plexus exploration involves exposing the
and that the fibrin sealant is thawed and in the room.
actual plexus through an incision in the crease of
A fibrin sealant (which consists of human fibrinogen
the neck to localize the extent of the lesions. A
and human thrombin) is utilized as an off-label
distinction is made between nerve root avulsion,
adjunct to suturing the nerve fascicles together. The
neurotmesis or rupture of nerve roots, or axonotmefibrin sealant takes approximately 60 to 110 minutes
sis based on inspection of the nerves and electrical
to thaw at room temperature, and unopened pouches
22 OR Nurse 2013 March
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stimulation with a nerve stimulator.9 The most common finding in brachial plexus injuries is a neuromain-continuity of the upper trunk located at the C5
and C6 nerve roots.8 A neuroma-in-continuity is a
lesion where the intraneural architecture of a nerve
is severely damaged, but the nerve is not severed
into two separate parts. A nerve root is considered
to be avulsed if a dorsal root ganglion is visible,
neuroma formation is absent, and there is an absence
of muscle contractions in response to stimulation.
Neurotmetic nerves are normal in appearance at the
intraforaminal level, have an increase in diameter of
the nerve, abundant fibrosis, and provide weak
muscle contractions when stimulated. Axonotmetic
nerves have no increase in diameter, limited fibrosis,
and provide a muscle contraction that induces limb
movement.9,14 These findings determine which
type of repair is required to restore function to the
affected upper extremity.
The phrenic nerve is identified and stimulated to
assess diaphragmatic function and involvement.
Careful neurolysis of the phrenic nerve typically
occurs if it is involved in the neuroma and may result
in a weakening in the diaphragm function on the
affected side postoperatively. If a phrenic nerve
injury occurs, most resolve within 1 year (postoperatively) without intervention.15
Nerve grafting
Nerve reconstruction is recommended for neurotmetic nerves, which involves dissecting the neuroma
and stimulating the proximal stumps to determine if
a muscle contraction occurs. The quality of the muscle
Schematic of a brachial plexus
repair
contraction distal to the stimulus determines if the
nerve root is suitable for grafting. Some surgeons
send the dissected nerves to pathology for histologic
evaluation to determine if the proximal and distal
stumps are viable targets for grafting and reinnervation.
The sural nerve is a sensory nerve used for grafting
that runs down the posterior calf. It is dissected free
from surrounding attachments through 2 to 3 cm
transverse incisions starting at the lateral malleolus to
the popliteal crease. Additionally, 13 to 15 cm of
sural nerve can be harvested from each leg of a 10 kg
infant. Typically, the distance between the distal
and proximal stumps is 2.5 to 4.5 cm in length, and
segments of the grafts are placed as cables in a tensionfree manner.9,12 Several nerve graft segments are
utilized to bridge the gap between the stumps. The
fibrin sealant is used to coapt the grafts to the nerves
(see Schematic of a brachial plexus repair).
Nerve transfers
Nerve transfers are becoming more common in the
treatment of brachial plexus injuries. Transfers are
either used in conjunction with nerve grafting where
there are nerve root avulsions, neurotmetic nerves in
certain portions of the plexus, or in lieu of the reconstruction. Nerve transfers are a direct motor-to-motor
neural connection and are utilized to target particular
muscle groups to restore a function. For instance, one
common nerve transfer that is performed to restore
function of the infraspinatus muscle transferring the
terminal motor branch of the spinal accessory nerve
to the suprascapular nerve (see Nerve transfer: Spinal
accessory to suprascapular nerve transfer). This transfer
Nerve transfer: Spinal accessory
to suprascapular nerve transfer
C5
m
2c
SSN
C6
m
3c
2.5
cm
UT
7
ck C
s ne
Cros
LT
2.5 cm
Key: SSN = suprascapular nerve, UT = upper trunk, LT = lower trunk,
C5 = cervical level 5 nerve root, C6 = cervical level 6 nerve root,
C7 = cervical level 7 nerve root.
Art re-created and provided as a courtesy of the authors.
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Photo provided as a courtesy of the authors. Permission to use was
obtained at the time the photo was taken.
March OR Nurse 2013
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Restoring function for children with brachial plexus birth palsy
targets the child’s ability to externally rotate the
shoulder. In the case where four to five nerve roots
of the plexus are avulsed, a contralateral C7 transfer
may be performed to restore hand function. This
transfer involves “borrowing” C7 from the nonaffected side to tunnel it through the retropharyngeal
space, and connect it with sural nerve grafts to the
distal stump of the lower trunk. Other transfers
may include the utilization of intercostal nerves or
fascicles of the ulnar nerve to motor branches of
the biceps. This restores elbow flexion and/or
the long head of the triceps branch of the radial
nerve to the axillary nerve to improve shoulder
abduction.8
Postoperative care and concerns
At the conclusion of the case, all incisions are
irrigated with 0.9% sodium chloride and closed
with absorbable sutures in the deep dermis and the
subcuticular. A topical skin adhesive is also applied
to all of the incisions, and neck incisions are covered with gauze and transparent film dressing.
The legs are often placed in soft casts to protect
the integrity of the incisions. The upper extremity
is placed in a stockinette sling and elastic wrap with
an abdominal pad placed over the shoulder and
between the arm and torso for padding. The arm
is adducted and flexed against the anterior chest
(see Infant after surgery). The arm is maintained in
this position for 2 to 3 weeks. Typically, the infant
spends 1 to 2 days in the hospital after surgery.
Infant after surgery
Soft casts are applied to lower extremities to
protect leg incisions. Elastic wrap and stockinette dressing are applied to upper extremity
postoperatively.
Photo provided as a courtesy of the authors. Permission to use was
obtained at the time the photo was taken.
24 OR Nurse 2013 March
_
_
_
.
The infant is transported home in a car bed to
prevent restraining straps from disrupting the neck
incision and repair of the plexus.
Concerns to consider following a brachial plexus
repair are diaphragm function, signs of infection, and
pain. A repeat chest ultrasound is performed during
the first postoperative day and compared with the
preoperative evaluation to determine if there are any
differences. If the phrenic nerve was stunned during
the neurolysis, the diaphragm on the affected side
will show a weakening or paralysis on ultrasound.
The infant’s respiratory state should be monitored in
addition to the ability to feed appropriately. Infants
who have a phrenic nerve injury sometimes have
difficulty coordinating the suck, breathe, and
swallow pattern.
Infection and pain are common considerations
following any surgical intervention. Parents should be
educated regarding how to identify signs and symptoms of infection, determining and treating pain, and
caring for the incisions. Treating the child with acetaminophen may be sufficient in treating the pain.
The return of function in the affected extremity is
variable. The rate of recovery is determined by the
distance from the proximal stump to the neuromuscular junction.12 The first evidence of return consists
of improvements in shoulder movement and is seen
within 6 months (postoperatively) followed by a
flicker of biceps.16 The degree of return is monitored
by clinical exam at 3- to 6-month intervals. Further
recovery may be seen up until 3 to 4 years postoperatively when improvements in function plateau.12
During the recovery period, therapy and home
exercise programs are an important part of care so
that all joints remain supple and loose until the
child is able to move the joint independently.
Outcomes
The surgical outcomes for patients undergoing
primary nerve repair by 6 months of age at the
authors’ center have indicated that 54% don’t
require additional surgical intervention, and 64%
don’t require treatment with onabotulinumtoxinA
to correct muscle imbalances. Significantly, fewer
Narakas I patients require additional intervention as
opposed to Narakas IV patients, as expected, based
on the severity of initial injury. Nerve transfers
showed a higher increase in function pertaining to
the Toronto Score during 1-year follow-up as
opposed to nerve grafting alone in all Narakas levels.
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While there isn’t one appropriate treatment plan
for all patients with brachial plexus injuries, there
are many options available to treat each individual
child. The controversy regarding timing and appropriate treatment continues, as physicians performing
nerve repair surgeries continue to study and share
outcomes across the globe. OR
REFERENCES
1. Schmitt C, Mehlman CT, Meiss AL. Hyphenated history: Erb-Duchenne
brachial plexus palsy. Am J Orthop (Belle Mead NJ). 2008;37(7):356-358.
2. Foad SL, Mehlman CT, Ying J. The epidemiology of neonatal brachial
plexus palsy in the United States. J Bone Joint Surg Am. 2008;90(6):12581264.
3. Bae DS, Waters PM, Zurakowski D. Reliability of three classification
systems measuring active motion in brachial plexus birth palsy. J Bone
Joint Surg Am. 2003;85-A(9):1733-1738.
4. Narakas A. Obstetric brachial plexus injuries. In: Lamb D, ed. The
Paralysed Hand. Edinburgh: Churchill Livingstone; 1987:116-135.
5. Clarke HM, Curtis CG. An approach to obstetrical brachial plexus
injuries. Hand Clin. 1995;11(4):563-580; discussion 580-581.
6. Foad SL, Mehlman CT, Foad MB, Lippert WC. Prognosis following
neonatal brachial plexus palsy: an evidence-based review. J Child Orthop.
2009;3(6):459-463.
9. Malessy MJ, Pondaag W. Obstetric brachial plexus injuries. Neurosurg
Clin N Am. 2009;20(1):1-14, v.
10. Waters PM. Comparison of the natural history, the outcome of
microsurgical repair, and the outcome of operative reconstruction in
brachial plexus birth palsy. J Bone Joint Surg Am. 1999;81(5):649-659.
11. Gilbert A. Long-term evaluation of brachial plexus surgery in
obstetrical palsy. Hand Clin. 1995;11(4):583-594; discussion 594-595.
12. Borschel GH, Clarke HM. Obstetrical brachial plexus palsy. Plast
Reconstr Surg. 2009;124(suppl 1):144e-155e.
13. U.S. Food and Drug Administration. Highlights of Prescribing
Information. TISSEEL [Fibrin Sealant]. http://www.fda.gov/downloads/
BiologicsBloodVaccines/BloodBloodProducts/ApprovedProducts/
LicensedProductsBLAs/FractionatedPlasmaProducts/ucm072968.pdf.
14. Pondaag W, van der Veken LP, van Someren PJ, van Dijk JG,
Malessy MJ. Intraoperative nerve action and compound motor action
potential recordings in patients with obstetric brachial plexus lesions.
J Neurosurg. 2008;109(5):946-954.
15. Xu WD, Gu YD, Lu JB, Yu C, Zhang CG, Xu JG. Pulmonary
function after complete unilateral phrenic nerve transection. J Neurosurg.
2005;103(3):464-467.
16. Hentz VR. Congenital brachial plexus exploration. Tech Hand Up
Extrem Surg. 2004;8(2):58-69.
Melissa Miller is a Registered Nurse II at Cincinnati Children’s Hospital
Medical Center in Cincinnati, Ohio. Allison Allgier is a Clinical Program
Manager at Cincinnati Children’s Hospital in Cincinnati, Ohio. Emily
Louden is an Outcomes Coordinator of the Brachial Plexus Center at
Cincinnati Children’s Hospital Medical Center in Cincinnati, Ohio.
7. Ramos LE, Zell JP. Rehabilitation program for children with brachial
plexus and peripheral nerve injury. Semin Pediatr Neurol. 2000;7(1):52-57.
The authors and planners have disclosed that they have no financial
relationships related to this article.
8. Hale HB, Bae DS, Waters PM. Current concepts in the management
of brachial plexus birth palsy. J Hand Surg Am. 2010;35(2):322-331.
DOI-10.1097/01.ORN.0000427258.62872.5c
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