Brachial Plexus Birth Palsy - Boston Children`s Hospital

Transcription

Brachial Plexus Birth Palsy - Boston Children`s Hospital
Brachial Plexus
Birth Palsy
Orthopedic Center
The Brachial Plexus Program, within the Orthopedic Center, is a national and international
referral center for children with brachial plexus birth palsy. Our brachial plexus team provides comprehensive care — from early nerve surgery, to early therapy, to later reconstructive orthopedic surgery and therapy if needed.
What is the brachial plexus?
The brachial plexus is a complex network of nerves between the neck and shoulders.
These nerves control muscle function in the chest, shoulder, arms and hands, as well as
sensibility (feeling) in the upper limbs.
Using a research and innovation driven approach, our program’s team of surgeons, nurses
and therapists have cared for more than 1,200 children with brachial plexus birth palsy.
What is brachial plexus birth palsy (BPBP)?
Brachial plexus birth palsy is an injury to the brachial
plexus nerves that occurs during childbirth. The
nerves of the brachial plexus may be stretched,
compressed, or torn in a difficult delivery. The result
might be a loss of muscle function, or even paralysis of the upper arm. Injuries may affect all or only a
part of the brachial plexus:
• Injuries to the upper brachial plexus (C5, C6)
affect muscles of the shoulder and elbow.
• Injuries to the lower brachial plexus (C7, C8 and
T1) can affect muscles of the forearm and hand.
Services provided through the Brachial Plexus Program include:
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microsurgical reconstruction
nerve grafts and transfers
tendon transfers
osteotomies
open reduction of shoulder and
elbow dislocations
arthroscopic surgical care
physical therapy
occupational therapy
parental and child support program
How common is brachial plexus birth palsy?
Brachial plexus birth palsies occur in about one to
three out of every 1,000 births.
How is brachial plexus birth palsy diagnosed?
Brachial plexus birth palsy can be diagnosed by
your baby’s pediatrician upon a thorough medical
history and physical examination. Since the majority of babies with a brachial plexus injury recover
in the first month to six weeks of life, these exams
can be scheduled with a primary care doctor.
Children who continue to have problems beyond
six weeks should be seen by an orthopedist or
brachial plexus specialist.
Peter Waters, MD
Clinical Chief, Orthopedic Center
Director, Brachial Plexus Program
Director, Hand and Orthopedic Upper
Extremity Program
John E. Hall Professor of Orthopedic Surgery,
Harvard Medical School
Donald Bae, MD
Assistant Professor of Orthopedic Surgery,
Harvard Medical School
Orthopedic Center
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Apurva Shah, MD, MBA
Instructor in Orthopedic Surgery,
Harvard Medical School
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300 Longwood Avenue
Illustration Copyright ~ 2011 Nucleus Medical Media,
All rights reserved. www.nucleusinc.com
In addition to a physical exam, doctors may perform special
imaging studies, like an MRI or nerve conduction studies.
These tests are not as reliable for babies as for adults, and they require anesthesia. If
accompanying fractures are suspected, doctors may take an x-ray. It’s important to find an
experienced doctor who will be able to track your child’s progress over repeated exams.
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Boston, MA 02115
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617-355-6021
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childrenshospital.org/bp
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Once my child is diagnosed with BPBP, how soon should we see the specialist?
Once your child’s pediatrician has made a diagnosis, it’s safe to wait up to four weeks for a
comprehensive evaluation by an orthopedist or specialist.
Patterns of Injury
C5-C6-C7 (formerly called Erb’s palsy)
This represents roughly 60 to 70 percent of BPBP injuries.
• it involves the upper portion (C5, C6, and sometimes C7) of the brachial plexus
• a child typically has weakness involving the muscles of the shoulder and biceps
• home physical therapy begins when a baby is 3 weeks old to prevent stiffness,
atrophy and shoulder dislocation
How often should my child be seen by her orthopedist after her initial appointment?
How often your child should be observed depends on her return of function. Typically, she
may need to be seen every one to three months until she is 6 months old, then every six
months through the time she’s 24 to 36 months old.
C5-T1 (total plexus involvement)
This represents roughly 20 to 30 percent of BPBP injuries.
What are the types of brachial plexus birth palsy?
Brachial plexus birth palsies are often categorized according to the type of nerve injury and
the pattern of nerves involved.
Horner’s syndrome
• this represents roughly 10 to 20 percent of injuries
• it is usually associated with an avulsion (a tear at the spinal cord)
• the sympathetic chain of nerves has been injured, usually in the T2 to T4 region
• the child may have ptosis (drooping eyelid), miosis (smaller pupil of the eye), and
anhydrosis (diminished sweat production in part of the face)
• the child may have a more severe injury of the brachial plexus
There are four types of nerve injuries
Stretch (neurapraxia)
• the nerve has been stretched but not torn—the injury occurs outside the
spinal cord
• it’s the most common form
• affected nerve(s) may recover on their own—usually within 3 months of the baby’s life
Klumpke’s palsy
• this almost never occurs in babies or children
• it involves the lower roots (C8, T1) of the brachial plexus
• it typically affects the muscles of the hand
Rupture
• the nerve is torn, but not where it attaches to the spine–the injury occurs
outside the spinal cord
• it’s a common form
• it may require surgical repair
Avulsion
• the nerve roots are torn from the spinal cord–the injury occurs at the spinal cord
• this is a less common form (roughly 10 to 20 percent of BP cases)
• it cannot be surgically repaired directly—damaged tissue must be surgically replaced
(nerve transfers)
• it can injure the nerve to the diaphragm, causing difficulty with breathing
• a droopy eyelid on the affected side may indicate a more severe injury
(Horner’s syndrome)
Neuroma
• the nerve has tried to heal, but scar tissue has formed and presses against the injured
nerve or interferes with nerve function
• it may require surgical treatment with nerve reconstruction and/or secondary
tendon transfers
Orthopedic Center
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300 Longwood Avenue
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617-355-6021
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childrenshospital.org/bp
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How is brachial plexus birth palsy treated?
Children’s Hospital Boston’s Brachial Plexus Program provides comprehensive
care–including evaluation, diagnosis, consultation, surgery, non-surgical therapies and
follow-up care, which may include:
Osteotomy
• procedure in which bones are cut
and reoriented
• may improve upper extremity function by better
positioning the hand and arm
• most commonly performed on the humerus (upper arm bone) or forearm
Observation
Most brachial plexus birth palsies will heal on their own. Your doctor will monitor your child
closely. Many children improve or recover by 3 to 12 months of age. During this time, ongoing exams should be performed to monitor progress.
Tendon transfers
• involves separating the tendon from its normal attachment and reattaching it to a new location
• extensive post-operative therapy
• done between 1 year of age and adulthood
• allows a healthy muscle to help a weaker or injured muscle perform its
desired function
• usually performed around the shoulder to improve the ability to raise the arm, but may
be used in forearm, wrist or hand
• patients usually in a cast for four to six weeks after surgery
• in some cases, shoulder weakness may cause limitations in motion that aren’t
amenable to tendon transfers
Physical therapy (and/or occupational therapy)
Therapy is recommended to help maximize use of the affected
arm and prevent tightening of the muscles and joints. With the
teaching and guidance of therapists, parents learn how to perform
range of motion (ROM) exercises at home with their child several
times a day. These exercises are important to keep the joints and
muscles moving as normally as possible.
Botox® injections
Botox® may be used (mainly for the shoulder) to:
• help with joint motion
• rebalance muscles
• prevent contractures and shoulder dislocations
Open reduction of the shoulder joint (capsulorraphy)
• reducing (placing the humeral head back in joint) and surgically tightening loose tissue
around the shoulder joint
• usually performed when persistent muscle weakness has caused shoulder joint instability or dislocation
• performed through a surgical incision -or- using arthroscopy
• often performed in conjunction with other surgical procedures
Surgery
Children who continue to have problems three to six months after
birth may benefit from surgical treatment. Your child’s doctors have
several surgical options for treating brachial plexus birth palsy, including:
Free muscle transfers
• typically using muscle (gracilis) from patient’s leg(s)
• extensive surgery requiring reconnection of blood vessels and nerves
under microscope
• used only when there are no local muscles in the arm or hand to replace
dysfunctional muscles
Microsurgery (10 to 20 percent of all BPBP surgery)
• recommended if recovery is still inadequate three to six months after birth
• to repair or reconstruct the injured nerves
• can be nerve grafts, usually from the leg (sural nerves) between nerve root and nerve
to muscle
• can be nerve transfers from other areas of the brachial plexus (or other areas of the
body): for more serious BPBP (avulsion)
• nerve reconstruction is best performed between 3 and 9 months of life and is usually
not beneficial for children beyond 1 year of age
Orthopedic Center
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What’s the prognosis if my child has brachial plexus
birth palsy?
The prognosis is dependent on the extent of the injury, and for
this reason, it varies from patient to patient. Most children achieve
normal or near-normal arm function without surgery. But not all
children recover fully. If a child does not recover fully, surgery can
improve her strength and/or motion and help optimize shoulder
joint development.
Ongoing brachial plexus research
The natural history of brachial plexus birth palsy remains
unknown, in part due to a lack of information on patients
evaluated from birth to adulthood. Based on what’s currently known about BPBP, it’s generally accepted that
microsurgery benefits infants:
• without recovery of biceps function by the age of 6
months, and
• with severe avulsion (a tear of the nerve at the spinal
cord) injuries by 3 to 6 months of age
One of the common problems with brachial plexus birth palsies can
be the abnormal development of the child’s shoulder joint, which can
happen over time. So, in addition to physical examinations, your child
may need ultrasound, magnetic resonance imaging (MRI) and/or computed tomography (CT) scans to monitor her shoulder development.
However, there’s significant controversy regarding the
Members of the Brachial Plexus Program team
ideal timing for microsurgery whose long-term outcomes
are unknown. The Brachial Plexus Program’s long-termTOBI (Treatment and Outcomes
of Brachial Plexus Injury) study of BPBP treatment is an international effort that includes
centers from North America, Europe and Australia.
Who will be on my child’s team?
Your child’s team may include her doctors, physical therapist, occupational therapist, mid-level provider (nurse practitioner and/or
physician’s assistant) and nurse, who will guide you through the treatment process. As
part of our family-centered approach, your child’s nurse will help with all your questions
and appointments. The nurse can also help you meet other families whose children have
undergone brachial plexus birth palsy treatment–in person and/or online.
Within Children’s Orthopedic Center, the Brachial Plexus Program and the Orthopedic Clinical Effectiveness Research Center (CERC) are doing extensive research on brachial plexus
birth palsy, including grant-funded research through the American Society for Surgery of
the Hand (ASSH) and the Pediatric Orthopaedic Society of North America (POSNA). This includes coordinating and analyzing data on brachial plexus patients from centers throughout
North America in the TOBI (Treatment and Outcomes Brachial Plexus Injuries) study.
Is there a support group for families of children with
brachial plexus birth palsy?
Yes, our Brachial Plexus Program offers support services,
and we encourage all families and children we treat to
participate in a parent/child support program.
The primary goal of this multi-center study is to determine the optimal age for microsurgical repair in infants with brachial plexus birth palsy and persistent upper extremity weakness. We’ll also compare the results of early microsurgery to those of secondary reconstructive surgery. This research is an effort to establish a standard of care at all hospitals,
and to determine the natural history (spontaneous recovery) and microsurgery results for
brachial plexus injuries. Our program is coordinating this multi-center TOBI study over the
next five years to determine the timing of microsurgery, tendon transfers and osteotomies.
We present all of our research papers on a national basis and publish these results in peer
review journals. Numerous papers have already been published and presented in these
areas and will continue to be published prospectively.
Please note: All patients with BPBP are invited to participate in this clinical study. During
your visit with us, you will be approached by a research coordinator to participate in this
and other studies—to help your child, you, and other children and parents determine what
is best for infants and children with brachial plexus injuries.
Laurie Travers, RN and Jess Burns, NP
Orthopedic Center
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childrenshospital.org/bp
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Brachial plexus terms
Open reduction shoulder joint— placing the humeral head back in the joint (glenoid)
and then surgically tightening loose tissue around the shoulder joint; can be performed
through surgical incision or using arthroscopy
Brachial plexus birth palsy (BPBP)— an injury (stretch, compression or tear) to all or part
of the brachial plexus nerve complex; occurs during childbirth; can result in loss of muscle
function or paralysis of upper arm
Osteotomy— controlled breaking or cutting and realigning of bone into correct position;
may improve upper extremity function; often used when shoulder weakness and/or joint
deformity cause limitations in motion that are not amenable to tendon transfers
Erb’s palsy— former name (sometimes still used) for one of the patterns of nerve injury in
BPBP—an injury to any or all of the C5-C6-C7 vertebrae; accounts for roughly 60 to 70 percent of BPBP injuries; typically results in weakness involving the muscles of the shoulder
and biceps
Physical therapy— a rehabilitative health specialty that uses therapeutic exercises and
equipment to help patients improve or regain muscle strength, mobility and other physical
capabilities
Free muscle transfer— a microsurgical option for treating BPBP that transfers muscle
tissue, usually from the gracilis muscle in the patient’s thigh, to the affected brachial plexus
area to restore flexion and extension functions in elbow, wrist and fingers
Range of motion (ROM) exercises— physical therapy exercises designed to improve or
restore flexion and extension of joints
Horner’s syndrome— one of the patterns of BPBP nerve injury; associated with an avulsion (see avulsion above); involves injury to the sympathetic chain of nerves; can indicate
more severe injuries of the brachial plexus
Reconstructive surgery— surgery performed to repair and/or restore a body part to
normal or as near normal as possible
Rupture— in BPBP, a tear of the nerve, but not where it attaches to the spine; can be
repaired surgically
Klumpke’s palsy— one of the patterns of BPBP nerve injury; involves injury to the lower
roots of the brachial plexus; almost never seen in babies and children
Stretch (neurapraxia)— a type of BPBP in which the nerve has been stretched but not
torn; the most common form; affected nerve may recover on its own
MRI (magnetic resonance imaging)— produces detailed images of organs and structures
within the body; shows the amount of damage to the brachial plexus
Microsurgery— surgery performed on extremely small structures or cells of the body using a microscope and other instruments
Tendon transfer— a surgical procedure that involves separating a tendon from its normal
attachment and reattaching it to a new location, often improving shoulder and wrist motion as well as elbow position and hand grip
Neuroma— scar tissue that has formed when a nerve has tried to heal; can interfere with
nerve function
Total plexus involvement— a BPBP of the C5-T1 vertebrae; accounts for roughly 20 to
30 percent of BPBP
Nerve conduction studies (NCS, nerve conduction velocity, NCV, electromyography,
EMG)— a two-part test consisting of nerve conduction studies (NCS) and electromyography (EMG). EMG can evaluate nerve disorders such as brachial plexus injuries (Erb’s palsies
and avulsion injuries).
“Waiter’s tip” position— a sign of BPBP; baby’s hand is turned away from the body
Nerve graft– a microsurgical procedure in which the damaged segment of an injured nerve
is removed, and a segment of nerve from the leg (usually sural) is attached to the remaining healthy section of the nerve
Nerve transfer— a microsurgical procedure in which the damaged segment of an injured
nerve is removed, and a segment of nerve from another area of the brachial plexus (or
another area of the body) is attached to the remaining healthy section of the nerve; often
used for avulsions (see avulsion)
Orthopedic Center
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300 Longwood Avenue
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Boston, MA 02115
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617-355-6021
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childrenshospital.org/bp
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Orthopedic Center
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300 Longwood Avenue
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Boston, MA 02115
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617-355-6021
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childrenshospital.org/bp
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