Approach

Treatment of brachial plexus birth injury (BPBI) has been evolving and remains controversial. Different centres recommend different treatments for different reasons, but the overall goal is to maximise the use and function of the affected extremity.

Newborns (<4 weeks) or infants (from 4 weeks to 1 year): initial treatment

Supportive care

  • After ruling out other causes of pseudoparalysis of the upper extremity, the arm is protected by careful handling of the infant.[46][47][48][49][86] The likelihood of potential re-injury during initial inflammatory response to the injury remains unclear.[47]

  • Instruct parents to avoid lifting the child under the arms and to lift him or her by supporting under the head and shoulders with one hand and under the buttocks with the other. When changing clothes, the affected arm should be placed into the sleeve first, followed by the head and the unaffected arm last. This avoids extremes of motion. When removing the clothes, the affected arm should be removed last. Normal bathing and infant care are otherwise instituted for the first 2 weeks.

  • In the past, safety-pinning the sleeve of the affected arm to the chest area was recommended, but this is no longer considered essential.

Physiotherapy or occupational therapy

  • Home stretches are typically initiated when the infant is 7 to 10 days old, unless there is a concomitant fracture. In that setting, stretches should begin at 3 to 4 weeks of age.[47][87][88][89] Parents are instructed in a home-based range-of-motion exercise programme, guided by weekly or bi-weekly formal therapy sessions.[46][47][48][49][86] Success is impacted by the degree of nerve injury and recovery.

  • Parents should perform gentle passive motion of all of the joints of the upper extremity at home at the nappy change, several times per day. Shoulder external rotation stretches are particularly important.

  • Arrange monthly follow-up in a formal clinic setting to monitor recovery and ensure range of motion is being maintained. Various scales (e.g., the Toronto Test Score, Active Movement Scale) are used to numerically monitor recovery of arm function.[48][53][54][55][90]

  • Most patients have relatively preserved hand and wrist function and develop reasonable elbow function; therefore, treatment is usually directed towards the shoulder.[46][47]​​​[49][86][91]​​​ Special attention is given to maintenance of the motion of shoulder abduction and external rotation to avoid contracture. Stretches to maintain passive supination are also recommended.

  • Splinting of the hand and wrist may be used to prevent contracture in children with hand and wrist involvement.

Specialty care

  • Early referral to a BPBI clinic is imperative for oversight of the infant’s care.[92] This facilitates serial examinations and timely surgical intervention if recovery is inadequate.[93][94]

Severe injury at birth and/or poor recovery in infancy

Indications for, and timing of, nerve reconstruction procedures for BPBI remain controversial.[89][94][95][96]​ Although certain instances clearly will require surgical intervention for any meaningful recovery to occur (nerve root avulsions or complete ruptures with no meaningful return of function), many children will have reasonable return of function on their own as time proceeds.[46][47][48][49][86][97][98]​ One systematic review concluded that the evidence that nerve reconstruction improves outcomes compared with conservative management is weak, relying on low-quality observational studies.[96]​ Some authors have also highlighted a potential risk that some outcomes (e.g., elbow flexion) may be worse following surgical treatment.[99]

Some authors advocate early surgery at 3 months of age if the patient has not shown meaningful return of biceps, shoulder, or hand function.[77][100][101][102][103][104]​ Other authors have shown that natural recovery may continue beyond 3 months of age, demonstrating good functional results without nerve reconstruction surgery.[6][64][105][106]

No standardised treatment can be recommended at this time. In one comparative study, functional outcomes following brachial plexus reconstruction were better than functional outcomes of children with spontaneous recovery at 5 months of age.[64] However, another study demonstrated improvements in function after late (after 9 months) brachial plexus reconstruction.[107] Irreversible changes to muscle and motor endplates occur after 18 to 24 months of denervation.[108] Nerve regeneration occurs at a rate of approximately 1 mm per day.[109] Surgical timing needs to take these time factors into account. The decision is based on the patient's recovery and the expertise and experience of the physician managing the patient's care. In general, children without full recovery by the age of 3 months are likely to have some residual impairment requiring ongoing treatment.[6][64][105][110]

For babies with global plexus injuries and little to no recovery of hand function, surgery is typically recommended around 3 months of age.[96][111][112] For children with Erb's palsy (C5/C6) and extended Erb's palsy (C5/C6/C7), surgery is recommended around 4 to 6 months of age.[96][112] Nerve grafting (removal of the damaged portion of the nerve and replacement with a portion of nerve from another area of the body) is preferred over neurolysis (clearing scar tissue from the nerve).[22][113] Nerve transfers offer another option for functional restoration. Nerve transfers may be utilised in conjunction with nerve grafting or in isolation for patients who do not have proximal roots available for grafting or who present late.[111][114][115]

The home-based range-of-motion exercise programme must be continued after postoperative immobilisation is complete and must follow any range of motion restrictions based on the specific procedure performed.

Ongoing monitoring in childhood

Shoulder and upper extremity function is monitored with the modified Mallet classification, other range-of-motion measures, and Medical Research Council (MRC) strength assessment.[56]

Children with BPBI may have limb length and girth discrepancies.[116][117] The severity of the discrepancy correlates with the severity of the nerve injury.[117] Elbow flexion contractures also frequently occur in children with BPBI.[118] Although usually able to flex their elbow with good strength, patients can show difficulty with activities about the head, and will abduct their shoulder in order to touch their mouth. This is referred to as the 'trumpet sign' and demonstrates the lack of shoulder external rotation needed to perform these activities without shoulder abduction. The trumpet sign can also be associated with lack of forearm supination.[Figure caption and citation for the preceding image starts]: Trumpet sign (elevated elbow)Thomas Campbell by commission [Citation ends].com.bmj.content.model.Caption@1ff06417

Monitoring is carried out for evidence of posterior shoulder subluxation, as the external rotators of the shoulder (supraspinatus, infraspinatus, and teres minor) are more severely involved than the internal rotators of the shoulder (pectoralis major, latissimus dorsi, teres major, and subscapularis) in patients with Erb's palsy. Patients who do not recover enough external rotation strength to counteract the internal rotators often show decreased function of the shoulder and arm.[46][47][48][49][64][86][105]​​​[Figure caption and citation for the preceding image starts]: Lack of external rotation after brachial plexus injuryThomas Campbell by commission [Citation ends].com.bmj.content.model.Caption@4489fe82 Additionally, denervated muscles have been shown to grow more slowly than the typical muscles, which contributes to contracture formation in children with BPBI.[119][120]

Signs of impending posterior subluxation include a decrease in passive external rotation of the shoulder (especially in adduction), shortening of the humeral segment (acromion to antecubital fold), asymmetric soft-tissue folds on the arm, a deep and asymmetric axilla, and a palpable humeral head posteriorly at the shoulder.[52][121] Orthopaedic treatments are directed towards maintaining the motion and function of the shoulder, elbow, wrist, and hand.

Parents should continue the home-based range-of-motion exercise programme, guided by weekly or bi-weekly formal therapy sessions as needed.

Secondary interventions: shoulder function

Choices concerning the most appropriate surgical procedure are individualised and are made by the treating surgical specialist. In infants and young children whose glenohumeral joint reduces in external rotation, the shoulder internal rotators may be injected with botulinum toxin type A and a shoulder spica cast applied in external rotation.[94][111][122][123][124]​​ This provides a prolonged, static stretch of the internal rotators. The temporary weakening of the internal rotators allows time for further recovery of the external rotators. The goal is to have more balanced forces about the glenohumeral joint when the effects of the botulinum toxin type A dissipate. Botulinum toxin injection is most likely to be successful in patients who still have at least 30 degrees of passive shoulder external rotation.[124] However, most available evidence is low quality, and additional studies are needed.[122][123]

Children under the age of 5 with posterior shoulder subluxation or dislocation can be treated with muscle lengthening (pectoralis major and subscapularis), muscle transfers (latissimus dorsi and teres major), and/or joint reduction.[125][126][127][128][129][130] Arthroscopic treatments have also been shown to be effective in improving shoulder function by release of the subscapularis tendon with and without latissimus dorsi and teres major transfer.[131][132][133][134][135] Similar findings have been noted following open glenohumeral release.[136] Some remodelling of the posterior glenoid and improved humeral head position can be expected, along with some remodelling of the humeral head.[66][125][127][136][137]

Additionally, functional capabilities will dramatically improve in overhead, at-the-head, and/or external rotation activities.[136][138][139][140][141][142][143][144] However, internal rotation is more limited as a result of these procedures, and deficits in midline function (toileting, zipping/buttoning trousers, and obtaining objects from the back pocket) are a potential complication.[131][140][145][146][147]

Patients older than 8 years with longer-standing shoulder dislocation or subluxation will not show remodelling and are often better served with an external rotation humeral osteotomy that will allow better arm position and function in a similar fashion to tendon transfers but with no attempt made to anatomically restore the integrity of the shoulder joint.[56][148][149]​​

Children aged between 5 and 8 may be indicated for any of these procedures depending on the severity of glenohumeral dysplasia and their clinical presentation. Ages 5 and 8 are not definitive age cutoffs, rather a general guide for which procedures are most commonly indicated in those age groups. The patient's active and passive range of motion, glenohumeral dysplasia severity, and patient-specific functional goals should all be considered in determining the most appropriate procedure.

Secondary interventions: elbow, forearm, wrist, and hand function

Choices concerning the most appropriate surgical procedure are individualised and are made by the treating surgical specialist.

Tendon transfers or other secondary procedures to other areas of the elbow, forearm, wrist, or fingers may be needed to improve function in these areas as well.[150][151][152]​​ Tendon transfers can be considered for children with an appropriate and expendable donor. Tendon transfer (unlike nerve surgery) is not time-sensitive, so can be considered as second-line for children who were not candidates for or who did not see significant improvements with nerve surgery, as long as passive motion is maintained or can be restored.

Patients who lack elbow flexion may be candidates for bipolar latissimus dorsi flexorplasty if they have a functioning latissimus dorsi.[153]

Management of residual deficits in the forearm is largely dictated by the passive range of motion. Supination deformity may be addressed with biceps rerouting if passive motion is maintained. If passive motion is limited, osteotomy of the radius or radius and ulna may be added to correct the deformity. One-bone forearm osteotomy is not as widely used, but may be an option for patients with severe deformity and lack of passive forearm rotation.[152]​Pronation deformity is generally better tolerated by patients than supination deformity.​

Lack of wrist extension may be treated with tendon transfer in some patients.[154] Many of the typical tendon transfer donors are not available in patients with BPBI, but flexor digitorum superficialis to the long and ring fingers may be transferred to the long finger metacarpal for restoration of wrist extension in patients with normal flexor digitorum superficialis and flexor digitorum profundus function.[154]​ Occasionally, wrist arthrodesis may be considered in skeletally-mature patients with no available donors for tendon transfer.

Botulinum toxin type A injections have also been used for the elbow flexors in infants with co-contraction of the elbow flexors and extensors.[155][156][157]​ Additionally, botulinum toxin injection and serial casting has been used to reduce elbow flexion contractures in older children.[118][122][123]

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