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    Shoulder Problems in ChildrenWith Brachial Plexus Birth Palsy:Evaluation and Management

    Abstract

    Traction injury to the brachial plexus sustained during the birth

    process that results in impaired neuromuscular function of the

    upper extremity continues to occur despite advances in modern

    obstetric care. The most common pattern of injury usually results in

    motor weakness of shoulder external rotation, leading to internal

    rotation contractures and subsequent deformity of the skeletally

    immature glenohumeral joint. Understanding of these deformities

    and effective surgical intervention have advanced greatly over the

    past decade. Restoration of balance between internal and external

    rotation forces around the shoulder has great potential for

    remodeling of the glenohumeral joint in the young child.

    Arthroscopic-directed release of the contracture, with select use of

    latissimus dorsi transfer to provide external rotation power, has

    proved to be effective for many children with these contractures.

    Epidemiologic studies show thatbrachial plexus palsy occurs in 1per 1,0001 to 4.6 per 1,000 births.2

    Conventional thinking that 80% to

    90% of children recover must be

    tempered by the understanding that

    there are differences in what is meant

    by recovery. A completely normal

    arm free of any sequelae is probably

    less common than often realized. In

    one study, persistent restriction in

    passive range of shoulder motion

    was observed in 54% of children

    who did not demonstrate complete

    neurologic recovery by 3 weeks, de-

    spite many of them going on to a

    good functional recovery.3 The

    number of such children who will

    have contractures requiring surgical

    treatment also varies from center to

    center, but in one cohort 20 of 74

    children referred in early infancy

    from an established registry were in

    this category.4 Likely twice this many

    have some degree of abnormal gleno-

    humeral anatomy on magnetic reso-

    nance imaging (MRI).5

    Despite advances in obstetric care,the incidence of brachial plexus birth

    palsy is increasing, speculated to be

    the result of increasing birth

    weights.1,2 The neurologic injury in

    newborns may involve the entire

    plexus but most often involves the

    upper trunk, with varying degrees of

    severity. Injuries may be transient,

    with nearly complete neurologic re-

    covery (ie, antigravity biceps and del-

    toid function usually observed by age

    2 months), or they may result in a

    permanently flail arm (usually in as-

    sociation with a complete plexus le-

    sion and avulsion of the cervical spi-

    nal nerve roots). For these two

    extremes, there is little controversy

    that the early-recovery group does

    not need surgical intervention and

    that the latter group will fare poorly

    Michael L. Pearl, MD

    Dr. Pearl is Shoulder and Elbow

    Surgeon, Kaiser Permanente, Los

    Angeles Medical Group, Los

    Angeles, CA, and Assistant Clinical

    Professor, Department of

    Orthopaedic Surgery, University of

    Southern California, Los Angeles.

    Supported in part by research grants

    from Kaiser Permanente Southern

    California and American Shoulder

    and Elbow Surgeons. Dr. Pearl or a

    member of his immediate family has

    received royalties from Zimmer.

    Reprint requests: Dr. Pearl, Kaiser

    Permanente, Los Angeles Medical

    Center, 4760 Sunset Boulevard, Los

    Angeles, CA 90027.

    J Am Acad Orthop Surg 2009;17:

    242-254

    Copyright 2009 by the American

    Academy of Orthopaedic Surgeons.

    Review Article

    242 Journal of the American Academy of Orthopaedic Surgeons

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    without it. When the nerve roots are

    avulsed, microsurgical options are

    limited to nerve transfers from unin-

    volved areas, such as the intercostal

    and spinal accessory nerves. The in-

    dications for neurosurgery on the

    plexus for injuries that are intermedi-

    ate to these two extremes remain

    controversial. Depending on themedical center, recommendations

    typically involve plexus exploration

    and grafting from 3 to 9 months fol-

    lowing birth.

    With or without nerve repair or

    transfer, internal rotation contrac-

    ture of the shoulder is the most com-

    mon problem requiring treatment in

    children with incomplete recovery3-10

    (Figure 1). This contracture results

    from an imbalance between the

    strength of the relatively unaffected

    internal rotators and the paralytic

    external rotators (primarily the in-

    fraspinatus). Untreated, it usually

    leads to progressive glenohumeral

    deformity characterized by posterior

    displacement of the humeral head on

    an increasingly dysplastic and de-

    formed glenoid. Treatment protocols

    vary widely, making it challenging to

    compare the literature and establish

    definitive indications for both early

    microsurgery and late secondary or-

    thopaedic procedures.

    Clinical Evaluation

    Assessing the motor function of the

    infant and young child is difficult.

    Electromyography has not been reli-

    able for most clinical investigations

    in this patient population. As a re-

    sult, the physical examination, with

    all its inherent limitations, is the

    mainstay of analysis. Fear of the ex-

    amination, inability to comprehend

    directions, and lack of coordination

    from undeveloped motor function in

    the very young patient all challenge

    the examiner trying to assess active

    range of motion (ROM) and

    strength. The examiner must engage

    the child in activities and then ob-

    serve motor function. Young chil-

    dren and especially infants can often

    be prompted to reach for objects

    overhead (eg, lollipop, shiny keys),

    providing an indication of active ele-

    vation (Figure 2). The effectiveness

    of similar maneuvers in other direc-

    tions is less predictable. By necessity,

    therefore, the examination only ap-

    proximates a complete motor exami-

    nation, depending on the childs age

    and ability to cooperate. Alternatives

    to the muscle grading systems that

    are commonly used for adults, such

    as the British Medical Council5-point scale, are necessary.

    The Hospital for Sick Children in To-

    ronto, Canada, introduced an exami-

    nation scale, the Active Movement Scale

    (AMS), to address the limitations in the

    British Medical Council system, specif-

    ically that it is incongruous to grade the

    strength of a weak muscle against grav-

    ity if it is not clear that the muscle can

    function with gravity eliminated.11

    Thus, the first four grades of

    strength in the AMS are devoted to

    achieving full ROM with gravity

    eliminated (Table 1). This system is

    particularly useful for infants under

    evaluation for potential neurologic

    surgery, because they are especially

    weak, rarely have contractures, and

    have motor grades that must be

    based on observation alone because

    they cannot comply with commands.

    Internal rotation contracture (20 of external rotation) in an 11-month-old child. A, Clinical photograph demonstratingthe degree of contracture with the arm at the side. B, Restriction of passive external rotation was confirmed underanesthesia at the time of surgery. (Reproduced with permission from Pearl ML, Edgerton BW, Kazimiroff PA, Burchette

    RJ, Wong K: Arthroscopic release and latissimus dorsi transfer for shoulder internal rotation contractures andglenohumeral deformity secondary to brachial plexus birth palsy. J Bone Joint Surg Am 2006;86:564-574.)

    Figure 1

    Michael L. Pearl, MD

    April 2009, Vol 17, No 4 243

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    Conversely, this system is a limited

    tool for measuring strength in the

    context of marked limitations in pas-

    sive ROM. When contractures are

    present, they are not assessed in this

    system, and grades of strength may

    not sufficiently represent motor func-

    tion. Furthermore, it is unclear that

    the ability to achieve full ROM with

    gravity eliminated is requisite for

    generating appreciable quantities of

    force in specific joint positions in all

    circumstances. For example, a mus-

    cle with a low score (ie, incomplete

    ROM with gravity eliminated) may

    be stronger in certain midrange posi-

    tions than muscles graded higher

    simply because they can achieve a

    full range.

    In an ongoing effort to standardize

    evaluations, Bae et al12 performed a

    reliability study of three major scor-

    ing systems: Mallet classification,

    Toronto Test Score, and Hospital for

    Sick Children AMS. Two trained ex-

    aminers twice evaluated 80 children

    with brachial plexus birth palsy. Re-

    sults were evaluated for intraob-

    server and interobserver reliability as

    well as test-retest reliability. Positive

    intraobserver and interobserver cor-

    relations were noted, and test-retestreliability was excellent. The authors

    concluded that the modified Mallet

    classification, Toronto Test Score,

    and AMS are reliable instrument s

    for assessing upper extremity func-

    tion in patients with brachial plexus

    birth palsy. It is not clear, however,

    how the findings of two examiners

    from the same institution translate to

    the findings of multiple examiners

    from different medical centers over

    long time periods. More important,the effectiveness and extent to which

    these scores reflect muscle function

    and recovery of the limb is not ad-

    dressed by measures of reliability.

    No perfect scale or score exists that

    is applicable to children of all ages un-

    der consideration for neurosurgical or

    orthopaedic intervention. Accordingly,

    investigators and clinicians treating af-

    fected children are limited to neurologic

    examination tools in combination with

    measures of passive and active ROM ofall upper extremity joints. With regard

    to active ROM, the assessment is by ne-

    cessity an approximation for young

    children who cannot reliably follow

    commands.

    Imaging Studies

    Ultrasonography,13,14 arthrography,6

    and MRI5,7,15-19 have all been used to

    study the morphology of the gleno-

    humeral joint in children with bra-

    chial plexus palsy. The exact role

    and relative advantages of each of

    these modalities is debatable, al-

    though most centers now favor MRI.

    Ultrasonography is real-time and

    noninvasive, but the level of detail is

    lower than that of other modalities.

    Arthrography offers more detail than

    Same child as in Figure 1 at the same presurgical consultationdemonstrating 120 of active elevation in reaching for her motherswristwatch. Note the effect of internal rotation contracture on the ability toreach.

    Figure 2

    Table 1

    The Active Movement Scale11

    Observation

    Muscle

    Grade

    Gravity Eliminated*

    No contraction 0

    Contraction, nomotion

    1

    Motion range 2

    Motion > range 3

    Full motion 4

    Against Gravity

    Motion range 5

    Motion > range 6

    Full motion 7

    * Active motion through a full range withgravity eliminated must be demonstratedbefore advancing to a score that denotesmotion through the range in the presenceof gravity.

    Reproduced with permission from CurtisC, Stephens D, Clarke HM, Andrews D:The active movement scale: An evaluativetool for infants with obstetrical brachialplexus palsy. J Hand Surg [Am] 2002;27:470-478.

    Shoulder Problems in Children With Brachial Plexus Birth Palsy

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    ultrasonography but is invasive;

    however, it can be done at the time

    of surgical intervention, under the

    same anesthesia (Figure 3). MRI of-

    fers the most detail and potential for

    standardization but are costly and

    often require general anesthesia inyoung children.

    Regardless of the imaging modality

    used, it is difficult to justify any clini-

    cal study that does not assess the sta-

    tus of glenohumeral development. It

    is now evident that the internal rota-

    tion contracture of the diseased

    shoulder commonly leads to gleno-

    humeral deformity. Posteriorly di-

    rected forces displace the humeral

    head in the same direction. In grow-

    ing children, skeletal changes ensue.A false articulation often forms on

    the posterior aspect of the glenoid

    that becomes progressively retro-

    verted, leading to a potential array of

    deformities that have been described

    as flat, biconcave, and convex (ie,

    pseudoglenoid). Various classifica-

    tion systems have been pro-

    posed.3,7,14,20,21 They have in common

    the fact that with increasing defor-

    mity, there is increasing posterior

    displacement of the glenohumeraljoint from its normally centered and

    concentric position, and the normal

    concave shape of the glenoid be-

    comes increasingly convex. In ad-

    vanced deformities, the humeral

    head articulates with the posterior

    aspect of the convex glenoid and be-

    comes increasingly misshapen and

    retroverted itself18 (Figure 4).

    Surgical Management

    A detailed review of the indications

    and techniques for microscopic sur-

    gical intervention for these injuries is

    beyond the scope of this discussion,

    but it is important to recognize that

    microsurgical options are applicable

    only to the most severe injuries; ap-

    ply only to patients in the first year

    of life, after which these options are

    thought not to be effective; and

    never result in complete neurologic

    improvement, always leaving resid-

    ual impairment. Thus, secondary or-

    thopaedic procedures are a potential

    consideration for all children with

    incomplete recovery, regardless

    whether they receive microsurgical

    intervention. For avulsion injuries,

    the prognosis for natural recovery is

    so poor that early (

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    and timing of microsurgical interven-

    tion, with recommendations ranging

    from age 3 to 9 months.24,25 There is

    no doubt that with increasing delayin return of biceps function less

    spontaneous recovery will occur. Wa-

    ters26 has shown that children who

    do not develop this ability by age 5

    months do not do as well under con-

    servative management as those who

    receive microsurgical nerve interven-

    tion (grafting or neurotization). Ef-

    forts continue to refine surgical

    indications, with some centers rec-

    ommending a more comprehensive

    evaluation that includes muscles

    other than the biceps to predict re-

    covery.27

    Discerning which neurologic le-

    sions will improve equally well with

    late secondary orthopaedic interven-

    tion and no early microsurgery, as

    opposed to early microsurgery fol-

    lowed later by secondary procedures,

    is difficult. No study to date can de-

    finitively claim that microsurgery in

    combination with secondary ortho-

    paedic procedures results in better

    outcomes than secondary ortho-

    paedic procedures alone, although

    much of current practice is predi-

    cated on this belief. Smith et al

    24

    pre-sented long-term follow-up of 22

    children who had no brachial plexus

    surgery and for whom biceps recov-

    ery was delayed until age 3 to 6

    months. These children demon-

    strated comparable function to that

    reported for children who had mi-

    crosurgical repair or grafting to the

    brachial plexus. Adding to the confu-

    sion is the wide array of secondary

    procedures that have been associated

    in published accounts with clinicalsuccess.

    Secondary Interventions

    Internal RotationContracture Release

    Internal rotation contractures and

    the resultant glenohumeral deformity

    have been documented in children as

    young as age 5 months.5 Treatment

    of the contracture can be undertakenat any time because it will not re-

    cover spontaneously, although a pe-

    riod of formal physical therapy may

    be required for the parents to accept

    this fact. Four soft-tissue procedures

    and one bony procedure constitute

    the majority of surgical experience

    over the past two decades: (1) ante-

    rior capsular release, Z-plasty

    lengthening of the subscapularis ten-

    don with or without transfer of mus-

    cles for external rotation;28 (2) pecto-

    ralis major release with transfer of

    the latissimus and teres major as ad-

    vocated by Hoffer and colleagues;29,30

    (3) subscapularis slide with and

    without a latissimus transfer as origi-

    nally described by Carlioz and Brahi-

    mi31 and recommended by Gilbert et

    al;32 and (4) arthroscopic release of

    the internal rotation contracture

    with or without latissimus trans-

    fer.10,33,34 These approaches variably

    combine some form of contracture

    release with or without a muscle

    transfer to augment external rotation

    power. For children with extensive

    glenohumeral deformity, the prevail-ing recommendation is an external

    rotational osteotomy of the humerus,

    rotating the arm into a more func-

    tional position of external rotation.35

    How can these seemingly disparate

    approaches be reconciled? One way

    is to recognize that the internal rota-

    tion contracture results from the loss

    of the normal balance between exter-

    nal rotation and internal rotation,

    principally because of infraspinatus

    weakness. Different types of proce-

    dures that restore muscle balance

    and stability by reducing internal ro-

    tation strength and/or augmenting

    external rotation strength may dem-

    onstrate effectiveness. Similarly, a

    bony procedure that increases exter-

    nal rotation will improve function. It

    is also likely that commonly used

    clinical scoring systems, most nota-

    bly the Mallet, are too crude to dis-

    tinguish the outcomes among these

    approaches.

    Formal Anterior Approach

    The first surgical releases described

    in the early 20th century by Fair-

    bank36 and later modified by Sever37

    used a traditional anterior deltopec-

    toral approach. To reduce the recur-

    rence rate of the internal rotation

    contracture and add external rota-

    tion power, most centers added some

    version of the LEpiscopo transfer of

    the latissimus dorsi and teres major

    tendons. Modified versions of these

    approaches are still the preferred

    method of treatment. Kirkos et al28

    reported a 30-year mean follow-up

    on 10 children who underwent a re-

    lease of the upper half of the pecto-

    ralis major and the entire subscapu-

    laris and anterior capsule, along with

    T2-weighted gradient-recalled echoMRI scan of the child in Figures 1and 3, demonstratingpseudoglenoid configuration. Theglenoid contour and scapularcenter line are enhanced with linetracing. (Reproduced withpermission from Pearl ML,Edgerton BW, Kazimiroff PA,

    Burchette RJ, Wong K:Arthroscopic release and latissimusdorsi transfer for shoulder internalrotation contractures andglenohumeral deformity secondaryto brachial plexus birth palsy. JBone Joint Surg Am2006;86:564-574.)

    Figure 4

    Shoulder Problems in Children With Brachial Plexus Birth Palsy

    246 Journal of the American Academy of Orthopaedic Surgeons

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    rerouting (transfer) of the latissimus

    dorsi and teres major tendons to the

    pectoralis major stump. Gains in ex-

    ternal rotation deteriorated over

    time, and five patients had signifi-

    cant degenerative changes of the gle-

    nohumeral joint.

    Among the concerns regarding theanterior approach were that it re-

    sulted in poor cosmesis and poten-

    tially led to anterior dislocation of

    the glenohumeral joint and/or func-

    tionally significant external rotation

    contractures. Several authors have

    modified this approach with an aim

    to address some of these issues. Zan-

    colli and Zancolli38,39 have long ad-

    vocated an anterior approach. Their

    clinical reports extensively describe

    indications and surgical technique

    but provide limited data analysis. To

    minimize cosmetic concerns, these

    surgeons use an incision in the skin

    lines from the coracoid to the axilla

    (Figure 5, A). The latissimus transfer

    is done by performing a step cut of

    the tendon insertion, rerouting the

    released tendon posteriorly, and se-

    curing it to the remaining latissimus

    tendon anteriorly (Figure 5, B and

    C). The subscapularis is released,

    and sometimes the pectoralis and

    teres major are released as well.

    These authors warn against releasing

    the anterior capsule. Satisfactory re-

    sults have been reported with this

    technique, with an increase of 50average abduction and 45 average

    external rotation.39 In the presence of

    an incongruent joint, which typically

    is seen in children older than age 3

    or 4 years, Zancolli and Zancolli39

    recommend a rotational osteotomy

    of the humerus and warn against any

    attempt to reduce a posterior sub-

    luxation surgically when the joints

    surfaces are already deformed.

    Recent reports by other authors

    who use an anterior approach and

    who warn against releasing the ante-

    rior capsule have acknowledged that

    despite their preoperative intentions,

    at surgery it was not possible to re-

    store external rotation and reduce

    the glenohumeral joint without re-

    leasing the capsule.40,41 In the series

    of van der Sluijs et al,40 15 of 19 pa-

    tients with internal rotation contrac-

    tures required release of the anterior

    capsule to achieve external rotation

    and reduce the glenohumeral joint.

    In addition to the presence of con-

    tracted anterior soft tissues, these au-

    thors postulate that in many cases

    excessive retroversion of the hu-

    merus obligates an external rotationcontracture once the glenohumeral

    joint is reduced. In such cases as

    these, Birch recommends an internal

    rotational osteotomy as part of the

    same surgery (70 of the 183 cases in

    his series41).

    Hoffer Modification of theLEpiscopo Procedure

    A common surgical approach em-

    ployed by many medical centers,

    originally devised by Hoffer, uses a

    cosmetic incision in the axillary

    crease to release the pectoralis major

    and transfer the combined tendons

    of the latissimus dorsi and teres ma-

    jor muscles to the posterior rotator

    cuff29,30 (Figure 6). Hoffer et al29 first

    reported on this technique in 11 pa-

    tients, who achieved an average gain

    Schematic illustration of anterior release, pectoralis transfer, and latissimus dorsi transfer as described by Zancolli and

    Zancolli.

    39

    A, Incision. B, Detachment of the subscapularis, pectoralis major, and latissimus dorsi. C, Rerouting of thelatissimus tendon and reinsertion of the pectoralis into the subscapularis tendon. (Reproduced with permission fromZancolli EA, Zancolli ER III: Reconstructive surgery in brachial plexus sequelae, in Gupta A, Kay SPJ, Scheker LR[eds]: The Growing Hand. London, England: Mosby, 2000, pp 805-823.)

    Figure 5

    Michael L. Pearl, MD

    April 2009, Vol 17, No 4 247

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    of 64 of abduction and 45 of exter-

    nal rotation at a minimum 2-year

    follow-up. A subsequent report 20

    years later presented similar success

    in another eight children.30 A conten-

    tion of these authors and others ad-

    vocating this procedure is that one

    should avoid releasing the anterior

    capsule and even the subscapularis

    for fear of anterior dislocation or

    creation of an external rotation con-

    tracture. However, recent reports

    from different centers that used this

    surgical approach have shown that

    despite improvements in shoulder

    function, this procedure did not im-

    prove the glenohumeral deformity

    present in many of the patients.9,42 It

    is likely that this surgical approach is

    most effective for external rotation

    weakness in the absence of a severe,

    firmly fixed internal rotation con-

    tracture and glenohumeral defor-

    mity.

    Release of theSubscapularis Origin

    Gilbert et al32 have long advocated

    an approach originated by Carliozand Brahimi31 in which the subscap-

    ularis origin is released and the mus-

    cle reflected distally (Figure 7, A). In

    a report on 65 patients followed

    more than 5 years after this pro-

    cedure, Gilbert et al32 noted an aver-

    age gain in external rotation of 70

    when children were operated on at

    younger than age 2 years provided

    that the joint was congruent and the

    humeral head was round. Children

    operated on after age 4 years did not

    show similar improvement. For these

    children, and those that failed an ear-

    lier release, these authors recom-

    mended transfer of the latissimus

    dorsi tendon to the posterolateral ro-

    tator cuff as well (Figure 7, B).

    Among the stated principles of this

    surgical approach is that it avoids re-

    leasing the anterior capsule and only

    Illustrations of partial pectoralis major release (A) and transfer of combined latissimus dorsi and teres major tendons tothe posterior rotator cuff (B) as described by Hoffer. (Reproduced with permission from Hoffer MM, Wickenden R,Roper B: Brachial plexus birth palsies: Results of tendon transfers to the rotator cuff. J Bone Joint Surg Am

    1978;60:691-695.)

    Figure 6

    Illustrations demonstrating subscapularis release (A) and transfer of theisolated latissimus dorsi tendon (B) as described by Gilbert et al.32

    (Reproduced with permission from Gilbert A: Obstetric brachial plexus palsy,in Tubiana R [ed]: The Hand. Philadelphia, PA: WB Saunders, 1993, vol 4,pp 592, 594.

    Figure 7

    Shoulder Problems in Children With Brachial Plexus Birth Palsy

    248 Journal of the American Academy of Orthopaedic Surgeons

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    partially weakens the subscapularis,

    preserving some of its function. In so

    doing, this technique clearly tips the

    balance between internal and exter-

    nal rotation strength in favor of the

    weak external rotators. In one series,

    however, there was inadequate re-lease of the capsular contracture in 5

    of 25 children, requiring an anterior

    approach at the same surgical set-

    ting.6 A recent long-term follow-up

    analysis noted that many of the func-

    tional gains observed in the early

    postoperative period deteriorated

    with time, resulting in significant

    functional limitations in adulthood,8

    a seemingly consistent observation of

    all reported methods in the few very

    long-term studies available.28

    Arthroscopic SubscapularisTenotomy and CapsularRelease

    In 2003, Pearl33 first reported on ar-

    throscopic release with promising

    early results (Figure 8). A subsequent

    report on 33 children confirmed the

    utility of this procedure.10 The surgi-

    cal protocol used in these studies fol-

    lowed in part the recommendationsof Gilbert et al32 in that young children

    (3 years) receive arthroscopic release

    only and older children receive a simul-

    taneous latissimus dorsi tendon trans-

    fer. Nineteen children in this series re-

    ceived a release only; 14 had a release

    combined with a latissimus dorsi trans-

    fer. At a minimum 2-year follow-up, the

    mean passive external rotation in-

    creased by 67 (P < 0.005) in the 15

    children with a successful arthroscopic

    release (Figure 9) and by 81 (P 3

    years) treated with simultaneous release

    and latissimus transfer maintained gains

    in external rotation. At follow-up, the

    four younger children who required de-

    layed latissimus transfer had a mean

    passive external rotation of 78 and

    showed no ill effects of delayed trans-fer. This differential approach to man-

    aging children younger than age 3 years

    with an isolated arthroscopic release re-

    mains the authors preferred method of

    managing these contractures.

    In the report by Pearl,33 arthro-

    scopic release not only improved ex-

    ternal rotation but also demon-

    strated remarkable remodeling of

    glenohumeral deformity when pres-

    ent before surgery. Follow-up MRI

    was available for 15 of 18 children

    with advanced pseudoglenoid defor-

    mities at the time of release or trans-

    fer. All but the three most severe

    deformities (12 of 15) showed nor-

    malization of the glenohumeral joint

    on follow-up MRI scan, as evidenced

    by increased sphericity of the hu-

    meral head, restoration of the gle-

    noid concavity, and centralization of

    the humeral head on the glenoid

    fossa (Figure 10).

    Normalization of glenohumeral

    anatomy may result from a range of

    surgical methods of contracture re-

    lease as long as external rotation is

    restored and preserved at follow-up.

    Pedowitz et al

    34

    showed some im-provement of glenohumeral align-

    ment on MRI immediately after

    arthroscopic reduction with the

    shoulder held in external rotation by

    a spica cast. At our center, we have

    observed remodeling of deformity by

    a variety of surgical methods, open

    anterior release, subscapularis slide

    Figure 11), and now with our cur-

    rent protocol of arthroscopic release

    (Figure 10). Hui and Torode20 also

    reported improved glenoid retrover-

    sion for 23 children at an average

    follow-up of 43 months after open

    anterior release. Most recently, Wa-

    ters and Bae43 reported on 23 chil-

    dren with 83% showing remodeling

    of deformity after open soft-tissue

    procedures that included open gleno-

    humeral joint reduction (ie, capsulor-

    rhaphy). This was in contrast to their

    Arthroscopic view of a rightshoulder from a posterior portaldemonstrating the level ofsubscapularis tenotomy with anelectrocautery device (arrow).BT = biceps tendon, HH = humeralhead. (Reproduced with permissionfrom Pearl ML: Arthroscopic

    release of shoulder contracturesecondary to birth palsy: An earlyreport on findings and surgicaltechnique. Arthroscopy2003;19:577-582.)

    Figure 8

    Active external rotation 2 yearsafter arthroscopic release in thesame child as in Figures 1, 3, and4. (Reproduced with permissionfrom Pearl ML, Edgerton BW,Kazimiroff PA, Burchette RJ, WongK: Arthroscopic release andlatissimus dorsi transfer forshoulder internal rotationcontractures and glenohumeral

    deformity secondary to brachialplexus birth palsy. J Bone JointSurg Am 2006;88:564-574.)

    Figure 9

    Michael L. Pearl, MD

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    earlier report on patients treated sim-

    ilarly who did not receive a capsular

    release and showed no improvement

    in glenohumeral deformity.9

    Procedures that tip the balance of

    the shoulder rotators toward exter-nal rotation, either by sacrificing in-

    ternal rotator strength or augment-

    ing external rotator power, will

    inevitably weaken internal rotation

    or diminish internal rotation range.

    A loss of internal rotation was ob-

    served with the arthroscopic ap-

    proach, as well.10 As reported,

    The ability to reach up the

    back was not measured preop-

    eratively, but it was clearly re-stricted at the time of follow-

    up, with the children only able

    to reach between the sacrum

    and L5 on the average.10

    Earlier reports in the literature de-

    scribing results from other tech-

    niques have not addressed the result-

    ant loss of internal rotation

    sufficiently to allow for meaningful

    comparison. The recent report by

    Waters and Bae,43 describing internal

    rotation using the Mallet scale hand

    to spine score, described a mean im-

    provement from a pre-operative 1 toa postoperative 2 (the ability to

    reach S1 postoperatively). From

    these two reports, it appears that

    both surgical approaches (arthro-

    scopic and open capsulorraphy) re-

    sult in the same amount of internal

    rotation.10,43 It is not clear why this

    was observed as a decrease in func-

    tion in the arthroscopic study and an

    increase following capsulorraphy,

    but the difficulties in examining mo-

    tor function in young children may

    play a role. One may conclude from

    the literature on the various tech-

    niques of contracture release and

    tendon transfer, as just discussed,

    that procedures that do not release

    the anterior capsule will less consis-

    tently restore external rotation and

    improve glenohumeral deformity

    than those that target the jointcapsule. Accordingly, historic con-

    cerns regarding release of the sub-

    scapularis and the anterior capsule

    must be tempered by the evidence

    that not doing so will not release allcontractures and will leave behind

    many posteriorly displaced, de-

    formed glenohumeral joints without

    the possibility of remodeling. The ar-

    throscopic release allows for select

    attention to the subscapularis and

    anterior capsule in contrast to the

    earlier open procedures that required

    release of multiple superficial struc-

    tures as well.

    Rotational Osteotomy

    For older children or those with ad-

    vanced glenohumeral deformity, the

    prevailing recommendation has been

    to avoid soft-tissue procedures at the

    joint in favor of rotational osteot-

    omy of the humerus. The utility of

    this procedure was confirmed in a re-

    cent report by Waters and Bae.35

    These authors reported on 27 pa-

    A, Preoperative T2-weighted gradient-recalled echo MRI scan of a 4.7-year-old patient revealing mild pseudoglenoid. B, T2-weighted gradient-recalledecho MRI scan of the same patient 2 years after arthroscopic release and

    latissimus dorsi transfer, demonstrating remodeling to a concentric joint witha round humeral head well centered on the glenohumeral joint. (Reproducedwith permission from Pearl ML, Edgerton BW, Kazimiroff PA, Burchette RJ,Wong K: Arthroscopic release and latissimus dorsi transfer for shoulderinternal rotation contractures and glenohumeral deformity secondary tobrachial plexus birth palsy. J Bone Joint Surg Am 2006;86:564-574.)

    Figure 10

    T2-weighted gradient-recalled echoMRI scan taken 3.5 years

    postoperatively of a child who hada pseudoglenoid prior to extra-articular subscapularis slide at age2 years. The scan shows a roundhumeral head well centered on aconcave glenoid.

    Figure 11

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    tients who underwent rotational os-

    teotomy with plate fixation and

    achieved an average improvement of

    64 in external rotation and im-

    proved 5 points on a modified Mal-

    let scale. No clear guidelines exist,

    however, as to what age or degree ofdeformity should dictate this form of

    surgical treatment. As it is now evi-

    dent that many children younger

    than age 6 years are capable of sub-

    stantial remodeling, the decision to

    perform an osteotomy is all the more

    complex. Humeral osteotomy does

    improve the functional position of

    the hand, but it leaves the shoulder

    in a posteriorly dislocated position

    and eliminates all hope of remodel-

    ing. At present, the surgeon mustmake a judgment call regarding the

    growth potential of the child, the se-

    verity of the deformity, and her or

    his own surgical abilities in deciding

    to perform a soft-tissue procedure

    that recenters the humeral head on

    the glenoid fossa (Figure 12).

    Other Problems

    Poor Active Elevation

    Weakness of elevation is functionally

    limiting when active elevation is

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    Scapular dyskinesia may exist even

    when the internal rotation contrac-

    ture is not severe enough to compel

    surgical treatment. Although some

    weakness of the rhomboid and serra-

    tus anterior muscles may contribute

    to this scapular dyskinesia, these

    muscles are usually functional. The

    asymmetric scapular motion more

    likely relates to persistent stiffness of

    the glenohumeral joint and learned

    patterns of movement. As with a fro-

    zen shoulder, the ROM in these neu-

    rologically damaged shoulders is glo-

    bally restricted. Tightness in the

    superior structures such as the su-

    praspinatus will result in an abduc-

    tion contracture of the humerus rela-

    tive to the scapula. In severe cases, as

    the child lowers the arm to the side,

    the scapula will elevate. With move-

    ment, as the child tries to position

    the hand in space,38,39 the scapula

    will be carried along in an asymmet-

    ric manner. This abnormal scapular

    movement does not represent a mo-tor problem of the scapular muscles,

    but is secondary to changes at the

    glenohumeral joint in the most ex-

    treme cases.

    The acronym SHEAR has been

    used to refer to the deformity of

    scapular hypoplasia, elevation, and

    rotation.45,46 Nath and colleagues45,46

    postulate that this deformity is the

    cause, not the result, of the ensuing

    medial rotation contractures and gle-

    nohumeral deformity. They offer a sur-

    gical protocol aimed at correcting this

    root cause. Although this approach

    does focus interest on a perhaps ne-

    glected concern of the parent or guard-

    ian (ie, unusual scapular motion), its

    theoretical premise conflicts with much

    of our current understanding of the

    pathophysiology affecting the shoulder

    in brachial plexus birth palsy. Brachial

    plexus lesions have much less effect on

    the muscles that move the scapula (and

    none on the trapezius and levator scap-ulae) than on the muscles that exter-

    nally rotate the shoulder. It is incongru-

    ous to attribute an internal (medial)

    rotation contracture to aberrant scap-

    ular motion when the more proximate

    cause is readily evident.

    External RotationContractures

    External rotation contractures pre-

    sent a smaller subset of the problems

    encountered from birth palsy than

    internal rotation contractures, with

    14% reported by Zancolli and Zan-

    colli.38 This contracture does not re-

    sult in posterior displacement of the

    glenohumeral joint or in significant

    deformity, but it can be functionally

    disabling. External rotation contrac-

    ture may also occur iatrogenically

    following release of internal rotation

    contractures. Surgical intervention is

    indicated when functional limita-

    tions in personal care are severe and

    the child has great difficulty reaching

    the midline. Zancolli and Zancolli39

    described surgical release and length-ening of the posterior-superior rota-

    tor cuff for this contracture. Few

    others have experience with this ap-

    proach, and caution is advised before

    further weakening important motors

    of external rotation and elevation in

    affected children. However, internal

    rotational osteotomy of the humerus

    has been effective in helping appro-

    priately selected children both cos-

    metically and functionally in terms

    of their ability to reach the midlineto perform activities of personal

    care.10,41

    Summary

    Traction injuries to the brachial

    plexus during the birth process result

    in residual orthopaedic problems

    that most commonly affect the

    shoulder. Although the role of micro-

    surgical intervention has become

    clearer and the techniques refined,

    complete restoration of neuromuscu-

    lar function remains elusive. For

    motor weakness that follows the

    neurologic injury, internal rotation

    contractures most commonly occur

    as a result of external rotation weak-

    ness. This leads to deformity of the

    glenohumeral joint (ie, glenohumeral

    dysplasia, posterior humeral head

    subluxation). As a result, secondary

    orthopaedic evaluation and correc-

    tive procedures are an integral part

    of the treatment of these children.

    Internal rotation contractures must

    be managed aggressively to avert and

    even correct deformity of the gleno-

    humeral joint. A variety of surgical

    approaches to restore balance be-

    tween external rotation and internal

    rotation power has demonstrated

    Postoperative active elevation of140 (20 gain from thepreoperative state) 2 years afterarthroscopic release in the samechild as shown in Figures 1, 3, 4,and 9. Note the improved ability toreach, with improved externalrotation.

    Figure 13

    Shoulder Problems in Children With Brachial Plexus Birth Palsy

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    clinical success. Currently, clinical

    studies do not definitively favor one

    approach, but existing evidence sup-

    ports early correction of the internal

    rotation contracture and reduction

    of the glenohumeral joint by open or

    arthroscopic means, even if it re-quires release of the anterior capsule.

    Surgeons who have experience with

    both open and arthroscopic methods

    usually find that the arthroscopic-

    guided release allows for equal if not

    greater contracture release, with re-

    duced morbidity. Regardless of the

    method used for anterior capsule re-

    lease, most surgeons who treat bra-

    chial plexus birth palsy favor supple-

    menting the release with a transfer of

    the latissimus dorsi tendon, whetherin all children or in children older

    than age 3 years.

    Consideration of any of the proce-

    dures to improve shoulder function

    should include a realistic assessment

    of the childs ability to use the hand

    and of the limitations at the elbow. A

    nonfunctional hand is unlikely to be

    more useful even when it can be

    placed in a functional position. Simi-

    larly, an elbow that cannot extend

    because of a persistent flexion con-tracture or an absent triceps may im-

    pede a childs reach to a far greater

    extent than limitation of shoulder

    motion.

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