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: • • • • • • • • • • 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 2 Apurva Shah, MD, MBA Instructor in Orthopedic Surgery, Harvard Medical School | 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. | Boston, MA 02115 | 617-355-6021 | childrenshospital.org/bp 3 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 4 | 300 Longwood Avenue | Boston, MA 02115 | 617-355-6021 | childrenshospital.org/bp 5 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 6 | 300 Longwood Avenue | Boston, MA 02115 | 617-355-6021 | childrenshospital.org/bp 7 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 8 | 300 Longwood Avenue | Boston, MA 02115 | 617-355-6021 | childrenshospital.org/bp 9 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 10 | 300 Longwood Avenue | Boston, MA 02115 | 617-355-6021 | childrenshospital.org/bp 11 Orthopedic Center 12 | 300 Longwood Avenue | Boston, MA 02115 | 617-355-6021 | childrenshospital.org/bp 13