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Disease/ Disorder

Definition

Traumatic brachial plexopathy (TBP) is a neurological deficit, most commonly weakness, to either all or portions of an involved limb that is secondary to a traumatic injury to the brachial plexus.

Etiology

TBP is due to a traumatic injury to the brachial plexus. Traumatic injuries usually affect individual or multiple trunks, cords, or roots. It may result from violent stretching, penetrating wounds, or direct trauma.1

Epidemiology including risk factors and primary prevention

Although exact data is lacking, TBP usually affects young healthy adults and in particular, males.2 Children are susceptible to many forms of trauma which include motor vehicle accidents, falls, sports injuries, and assault. Injuries in which the head is pushed away from the shoulder typically result in upper plexopathies while injuries in which the arm and shoulder are pulled up typically result in lower plexopathies. A common upper plexopathy injury in American football is a “stinger” in which a player can experience a burning pain in the shoulder with transient weakness after a blow to the head, neck, or shoulder.3

Patho-anatomy/physiology

  •  All major nerves in the upper extremity originate from the brachial plexus
  • The brachial plexus arises from the C5-C8 and T1 ventral rami. These roots then join to form the subsequent trunks (lower, middle, upper), divisions (anterior, posterior), cords (lateral, posterior, medial), and terminal branches (musculocutaneous, radial, axillary, median, ulnar).
  • Traumatic injuries make localization difficult due to the large number of possible types and patterns of injury. Patients can have a variable degree of sensory and/or motor deficits in the upper extremity.
  • Types of injury based on Seddon’s classification of nerve injury include: neuropraxia, axonotmesis, or neurotmesis

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)

Seddon’s classification is a common way of classifying nerve injuries. There are three types of nerve injuries: 1) neuropraxia, 2) axonotmesis, 3) neurotmesis. Neuropraxia is a local injury to the myelin with the axon intact. Axonotmesis is an injury to the myelin and axon with the neural connective tissue intact. Neurotmesis is an injury to the myelin, axon and neural connective tissue.

As compared to axonotmesis or neurotmesis, neuropraxia has a much more favorable prognosis because the axons themselves are not injured. Axonotmesis results in Wallerian degeneration which leads to prolonged recovery and a less favorable prognosis overall.4 Neurotmesis is the most severe form and surgical repair is typically needed.

Specific secondary or associated conditions and complications

  • Horner’s syndrome is often associated with a C8-T1 injury.
  • There is an increased risk for contractures given the imbalance of healthy muscle groups versus affected muscle groups.
  • Concomitant injury of the phrenic nerve and the brachial plexus can occur 10-20% time.5

Essentials of Assessment

History

 Patients will report weakness, pain, and paresthesias of the affected limb. Key aspects include:

  • The mechanism of the trauma:  Supraclavicular/more proximal injuries commonly affect the trunks/root level while infraclavicular/more distal injuries commonly affect the cord/branches level.
  • Direction of traumatic force: Understanding whether force was superior or inferior can be helpful in localization and prognostication as well.
  • Distribution of sensory changes: Helpful in identifying specific dermatomal patterns.

Physical examination

Initial assessment should include a thorough neuromuscular exam focusing on strength, sensation, and reflexes.6

  • Upper trunk injuries (C5-C6) present as weakness in shoulder abduction, external rotation, elbow flexion, and wrist extension with sensory changes in the lateral arm/forearm. Biceps and brachioradialis reflexes are abnormal.
  • Lower trunk injuries (C8-T1) and medial cord injuries are nearly identical and present as weakness in the hand muscles with sensory changes in the medial arm, forearm, and hand. Notable exception for medial cord injury is sparing of C8 radial fibers.
  • Lateral cord injuries present as weakness in elbow and shoulder flexion, wrist flexion, and arm pronation with sensory changes in the lateral forearm and lateral hand. The biceps reflex is abnormal.
  • Posterior cord injuries present as weakness in shoulder abduction and adduction, elbow extension, and wrist extension with sensory changes in lateral arm, posterior arm/forearm, and dorsal hand. The brachioradialis and triceps reflexes are abnormal.
  • Pending the mechanism of trauma, examination could find any combination of the above.

Clinical functional assessment: mobility, self-care cognition/behavior/affective state

Patients will have limited mobility in the affected extremity. For infants and children, it is important for assessments to focus on how impaired mobility affects development. As they get older, the clinician will focus more on how it affects their self-care, activities of daily living, play, and other functional activities.

Laboratory studies

Routine laboratory studies are not needed for TBP.

Imaging

Magnetic Resonance Neurography (MRN) is becoming an increasingly popular tool for TBP assessment. It is predominantly used for preoperative planning but can also be used when EMG or conventional MRI findings are inconclusive. MRN is safe, well tolerated, and under most circumstances can accurately characterize the severity and location of nerve injury. It is most commonly utilized in identifying patients with root avulsions or other pre-ganglionic injuries who ultimately will need surgery.7

Supplemental assessment tools

Nerve conduction studies and electromyography (NCS/EMG) are the most widely used diagnostic tool for TBP but are not well-tolerated in the pediatric population. Sensory nerve action potentials (SNAPs) can be helpful in assessing pre-ganglionic versus post-ganglionic injury while SNAPs and compound motor action potentials (CMAPs) can assess neuropraxia versus axonal degeneration. SNAPs would be intact in a pre-ganglionic injury (such as a root avulsion) versus a postganglionic injury (such as plexopathy) where they would be abnormal.  Conduction block on CMAP and/or SNAPs suggests neuropraxia whereas decreased amplitude suggests axonal degeneration.  EMG can provide information about denervation and prognostic information regarding reinnervation.2 Overall, the lesion can likely be localized to specific root(s), trunk(s), or cord(s) involvement. The ideal timing to do NCS/EMG is at least 3 weeks out from injury.

Early predictions of outcomes

Indicators of poor prognosis include complete plexopathy, root avulsions, and neurotmesis. Neuropraxic injuries are associated with a much better prognosis and often make a full recovery.

Environmental

While most of these injuries cannot be prevented, parents and providers should optimize the safety of their patients in regards to equipment, situational awareness, and proper techniques in sports (e.g. tackling technique in American football). 

Social role and social support system

TBP can be a very debilitating injury and thus, a good support system is key for optimal recovery.  It is important to involve social work early on in the process to assist families in providing access to local and national support groups.  School accommodations may be needed to provide a 504 or individualized assessment plan (IEP) depending on the extent of injury, especially if there is presence of other trauma (traumatic brain injury, spinal cord injury, etc.)  In addition, the provider may need to inquire if the pediatric patient has concerns with body image and adequate peer support following injury.

Professional Issues

  • Athletics and stingers: Currently, there is no consensus on return to play after sustaining a stinger. The general consensus is the player should have complete resolution of symptoms with full motor strength and full painless cervical range of motion before returning to play.8
  • Driving: If the patient was driving prior to the injury, the provider will need to navigate prognosis, recovery, and safety to return to driving.  Local resources may be available to provide classes and therapies for driving; however, there may be challenges to insurance coverage and cost.  Transportation issues to school may need to further collaboration with social work and/or school.
  • School: School accommodations may be needed including a 504 or IEP to provide appropriate support.  This may include hand-writing modifications (especially if the dominant hand is involved), access to laptop, etc.

Rehabilitation Management and Treatments

Available or current treatment guidelines

Pediatric TBP injuries are exceedingly rare, so published literature describing treatment strategies is lacking. With no established consensus on the best treatment approach, therapeutic strategies are extrapolated from the adult population.9 Goals of treatment include motor and sensory recovery to restore upper extremity function and preventing muscle atrophy/contracture.10 This is accomplished through a combination of rest, physical therapy, and surgical intervention depending on the location and extent of injury.8 Prognosis is generally favorable with appropriate intervention.10

At different disease stages

Therapeutic approaches are largely guided by the type of nerve injury as defined by Seddon’s classification.10

Treatment of neuropraxia and axonotmesis injuries is primarily non-surgical with self-resolution within weeks to months. Stretching maintains flexibility and slows down atrophy.10 Physical therapy may be useful depending on the extent of neurological damage.8 Electrical stimulation, multivitamin supplements and neurotrophic agents have been reported useful, however lack supporting evidence and efficacy remains unclear.10

Surgery is considered when there is unequivocal evidence of neurotmesis or an absence of clinical/EMG recovery within 12 weeks after trauma. Priorities of surgery focus on restoration of shoulder abduction and elbow flexion but may also target hand function, particularly in younger patients11. Interventions include nerve graft, nerve transfer, or muscle transfer. Nerve transfers (commonly spinal accessory to suprascapular, radial to axillary nerve or ulnar to musculocutaneous) are frequently used given the predominance of pre-ganglionic injuries.8,9 Nerve grafts are less commonly used. Studies suggest both nerve grafting and nerve transfers offer equally good results. Free functioning muscle transfer (FFMT) techniques, most often gracilis to the upper arm, remain controversial given the uncertainty of its impact on growth and contracture formation.12 Muscles denervated for longer than 6 months prior to repair fail to fully recover from denervation atrophy, demonstrating a decrease in number (by 50%) and size of muscle fibers.13

Coordination of care

TBP injuries are suited for a multidisciplinary approach with the physiatrist collaborating with physical therapist, occupational therapist, and surgical specialist to devise and execute the most appropriate, individualized approach.  

Patient & family education

Prognosis is generally good with the majority of cases improving without surgical intervention8 and no relation of motor outcome and timing of surgical intervention when necessary.11

In contact sports, specifically football, players should be counseled on safe tackling technique and strengthening of the neck and core musculature.8

Measurement of Treatment Outcomes including those that are impairment-based, activity participation-based and environmentally based

Due to the rare condition of traumatic brachial plexus injury in pediatrics, there is no general consensus on the best measurements of treatment outcomes.  Functional Independent Measure (FIM) or WeeFIM may provide insight to activities of daily living; however, it is not specific for traumatic brachial plexus injury in the pediatric population.  One can also derive treatment outcomes based on serial physical exams and NCS/EMG studies.  It should be noted that there are several measures intended for birth brachial plexus injury, but not necessarily TBP.

Translation into practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

Previously, additional orthopedic procedures were utilized including shoulder/wrist arthrodesis or upper limb amputation; however, these have largely fallen out of favor with more widespread use of alternative nerve and muscle transfer procedures.10

Cutting Edge/ Emerging and Unique Concepts and Practice

Because this condition is rare in children, there are limitations in research surrounding cutting edge concepts and practice.

  • Neuromuscular electrical stimulation is a rehabilitative tool that may be utilized for nerve regeneration; however, research in TBP is limited to case reports.10,14,15
  • Constraint-induced movement therapy is a therapeutic tool that has been shown to improve cortical mapping in children with hemiplegic cerebral palsy and can improve activities in bimanual tasks in children with neonatal brachial plexopathy; however, there is no research that has been complete in children with traumatic brachial plexopathies.16–18  This may be a feasible therapeutic regimen to improve activities of daily living and bimanual tasks in traumatic brachial plexopathy.

Gaps in the Evidence-Based Knowledge

There is no consensus with regards to investigations or management. The rarity of the diagnosis thwarts research and hence leads us to extrapolate from adult literature. MR neurography in pediatric TBP is limited7 and NCS/EMG, if available, is poorly tolerated. Multi-site collaboration and research is needed to enhance knowledge.

References

  1. Tharin BD, Kini JA, York GE, Ritter JL. Brachial plexopathy: a review of traumatic and nontraumatic causes. AJR Am J Roentgenol. 2014;202(1):W67-75.
  2. Smania N, Berto G, La Marchina E, et al. Rehabilitation of brachial plexus injuries in adults and children. Eur J Phys Rehabil Med. 2012;48(3):483-506.
  3. Cifu DX. Braddom’s Physical Medicine & Rehabilitation. 5th ed. (Cifu DX, ed.). Elsevier; 2015:925-926.
  4. Rubin DI. Brachial and lumbosacral plexopathies: A review. Clin Neurophysiol Pract. 2020;5:173-193.
  5. Pornrattanamaneewong C, Limthongthang R, Vathana T, Kaewpornsawan K, Songcharoen P, Wongtrakul S. Diaphragmatic height index: new diagnostic test for phrenic nerve dysfunction. J Neurosurg. 2012;117(5):890-896.
  6. David Preston BS, ed. Brachial Plexopathy 3rd. In: Electromyography and Neuromuscular Disorders. Elsevier; 2012:468-472.
  7. Mazal AT, Faramarzalian A, Samet JD, Gill K, Cheng J, Chhabra A. MR neurography of the brachial plexus in adult and pediatric age groups: evolution, recent advances, and future directions. Expert Rev Med Devices. 2020;17(2):111-122.
  8. Ahearn BM, Starr HM, Seiler JG. Traumatic Brachial Plexopathy in Athletes: Current Concepts for Diagnosis and Management of Stingers. J Am Acad Orthop Surg. 2019;27(18):677-684.
  9. Chim H, Kircher MF, Spinner RJ, Bishop AT, Shin AY. Reconstruction of pediatric brachial plexus injuries with nerve grafts and nerve transfers. J Hand Surg Am. 2014;39(9):1771-1778.
  10. Belviso I, Palermi S, Sacco AM, et al. Brachial Plexus Injuries in Sport Medicine: Clinical Evaluation, Diagnostic Approaches, Treatment Options, and Rehabilitative Interventions. J Funct Morphol Kinesiol. 2020;5(2). doi:10.3390/jfmk5020022
  11. Garg K, Sinha S, Mahapatra AK, Sharma BS. Microsurgical outcome in posttraumatic brachial plexus injuries in children. Childs Nerv Syst. 2014;30(5):919-923.
  12. Madura T, Doi K, Hattori Y, Sakamoto S, Shimoe T. Free functioning gracilis transfer for reanimation of elbow and hand in total traumatic brachial plexopathy in children. J Hand Surg Eur Vol. 2018;43(6):596-608.
  13. Fu SY, Gordon T. Contributing factors to poor functional recovery after delayed nerve repair: prolonged denervation. J Neurosci. 1995;15(5):3886-3895.
  14. Rich JA, Newell A, Williams T. Traumatic brachial plexus injury rehabilitation using neuromuscular electrical muscle stimulation in a polytrauma patient. BMJ Case Rep. 2019;12(12). doi:10.1136/bcr-2019-232107
  15. Gordon T, English AW. Strategies to promote peripheral nerve regeneration: electrical stimulation and/or exercise. Eur J Neurosci. 2016;43(3):336-350.
  16. Werner JM, Berggren J, Loiselle J, Lee GK. Constraint-induced movement therapy for children with neonatal brachial plexus palsy: a randomized crossover trial. Dev Med Child Neurol. 2021;63(5):545-551.
  17. Chen YP, Pope S, Tyler D, Warren GL. Effectiveness of constraint-induced movement therapy on upper-extremity function in children with cerebral palsy: a systematic review and meta-analysis of randomized controlled trials. Clin Rehabil. 2014;28(10):939-953.
  18. Hoare BJ, Wallen MA, Thorley MN, Jackman ML, Carey LM, Imms C. Constraint-induced movement therapy in children with unilateral cerebral palsy. Cochrane Database Syst Rev. 2019;4:CD004149.

Author Disclosure

Eric Weidert, DO
Nothing to Disclose

Kayla Williams, MD
Nothing to Disclose

Rajashree Srinivasan, MD
Nothing to Disclose

Kelli Chaviano, DO
Nothing to Disclose