Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

newborns and infants

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initial supportive care

The affected arm is protected by careful handling of the infant.[46][47][48][49] The likelihood of potential reinjury 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. The affected arm should be placed into the sleeve first when dressing, followed by the head and the unaffected arm last (avoiding extremes of motion). The affected arm should be removed last on undressing. Normal bathing and infant care are otherwise instituted for the first 2 weeks.

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home exercises, referral to specialty care, and physical or occupational therapy

Treatment recommended for ALL patients in selected patient group

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 program, guided by weekly or biweekly 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 diaper 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 toward the shoulder.[46][47]​​[49][86][91]​​ Special attention is given to maintenance of 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.

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

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nerve reconstruction + postoperative therapy

Treatment recommended for SOME patients in selected patient group

Indications for, and timing of, nerve reconstruction procedures for BPBI remain controversial.[89][94][95][96]​ While 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 standardized 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 palsy (C5/C6) and extended Erb palsy (C5/C6/C7), surgery is recommended around 4-6 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 utilized 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 program must be continued after postoperative immobilization is complete and must follow any range of motion restrictions based on the specific procedure performed.

ONGOING

following initial treatment

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monitoring and home exercises ± physical or occupational therapy

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, elbow flexion contractures, and can show difficulty with activities about the head.[116][117][118] 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][105]

Monitoring is carried out for evidence of posterior shoulder subluxation or dislocation. 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]

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

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orthopedic intervention

Treatment recommended for ALL patients in selected patient group

Choices concerning the most appropriate surgical procedure are individualized 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 onabotulinumtoxinA (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 onabotulinumtoxinA 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 remodeling of the posterior glenoid and improved humeral head position can be expected, along with some remodeling of the humeral head.[66][125][127][136][137]

Patients older than 8 years do not show remodeling and are often better served with an external rotation humeral osteotomy (better arm position and functionality as compared with tendon transfers), with no attempt to anatomically restore the integrity of the shoulder joint.[56][148][149]

Children between ages 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.

Primary options

onabotulinumtoxinA: consult specialist for guidance on dose

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Plus – 

orthopedic intervention

Treatment recommended for ALL patients in selected patient group

Choices concerning the most appropriate surgical procedure are individualized 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.

OnabotulinumtoxinA (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]

Primary options

onabotulinumtoxinA: consult specialist for guidance on dose

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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