Review Article

Shoulder Problems in Children With Brachial Plexus Birth Palsy: Evaluation and Management
Abstract
Michael L. Pearl, MD

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.

E
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.

pidemiologic studies show that brachial plexus palsy occurs in 1

per 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, despite 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 glenohumeral anatomy on magnetic resonance 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 recovery (ie, antigravity biceps and deltoid function usually observed by age 2 months), or they may result in a permanently flail arm (usually in association with a complete plexus lesion and avulsion of the cervical spinal 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

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Figure 1

Internal rotation contracture (20 of external rotation) in an 11-month-old child. A, Clinical photograph demonstrating the degree of contracture with the arm at the side. B, Restriction of passive external rotation was conrmed under anesthesia 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 and glenohumeral deformity secondary to brachial plexus birth palsy. J Bone Joint Surg Am 2006;86:564-574.)

without it. When the nerve roots are avulsed, microsurgical options are limited to nerve transfers from uninvolved areas, such as the intercostal and spinal accessory nerves. The indications for neurosurgery on the plexus for injuries that are intermediate to these two extremes remain controversial. Depending on the medical center, recommendations typically involve plexus exploration and grafting from 3 to 9 months following birth. With or without nerve repair or transfer, internal rotation contracture of the shoulder is the most common 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 infraspinatus). Untreated, it usually leads to progressive glenohumeral deformity characterized by posterior displacement of the humeral head on an increasingly dysplastic and deformed glenoid. Treatment protocols vary widely, making it challenging to
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compare the literature and establish definitive indications for both early microsurgery and late secondary orthopaedic procedures.

Clinical Evaluation
Assessing the motor function of the infant and young child is difficult. Electromyography has not been reliable for most clinical investigations in this patient population. As a result, the physical examination, with all its inherent limitations, is the mainstay of analysis. Fear of the examination, 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 observe motor function. Young children and especially infants can often be prompted to reach for objects overhead (eg, lollipop, shiny keys), providing an indication of active elevation (Figure 2). The effectiveness

of similar maneuvers in other directions is less predictable. By necessity, therefore, the examination only approximates a complete motor examination, 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 Council 5-point scale, are necessary. The Hospital for Sick Children in Toronto, Canada, introduced an examination scale, the Active Movement Scale (AMS), to address the limitations in the British Medical Council system, specifically that it is incongruous to grade the strength of a weak muscle against gravity 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.

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Figure 2

Same child as in Figure 1 at the same presurgical consultation demonstrating 120 of active elevation in reaching for her mothers wristwatch. Note the effect of internal rotation contracture on the ability to reach.

Table 1 The Active Movement Scale11 Observation Gravity Eliminated* No contraction Contraction, no motion Motion range Motion > range Full motion Against Gravity Motion range Motion > range Full motion Muscle Grade

0 1 2 3 4 5 6 7

* Active motion through a full range with gravity eliminated must be demonstrated before advancing to a score that denotes motion through the range in the presence of gravity. Reproduced with permission from Curtis C, Stephens D, Clarke HM, Andrews D: The active movement scale: An evaluative tool for infants with obstetrical brachial plexus palsy. J Hand Surg [Am] 2002;27: 470-478.

Conversely, this system is a limited tool for measuring strength in the context of marked limitations in passive ROM. When contractures are present, they are not assessed in this system, and grades of strength may not sufficiently represent motor function. 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 muscle with a low score (ie, incomplete ROM with gravity eliminated) may be stronger in certain midrange positions 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 scoring 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. Results were evaluated for intraobserver and interobserver reliability as well as test-retest reliability. Positive intraobserver and interobserver correlations were noted, and test-retest reliability was excellent. The authors concluded that the modified Mallet classification, Toronto Test Score, and AMS are reliable instrument s for assessing upper extremity function 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 addressed by measures of reliability. No perfect scale or score exists that is applicable to children of all ages under consideration for neurosurgical or orthopaedic intervention. Accordingly, investigators and clinicians treating affected children are limited to neurologic examination tools in combination with measures of passive and active ROM of all upper extremity joints. With regard to active ROM, the assessment is by necessity 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 glenohumeral joint in children with brachial plexus palsy. The exact role and relative advantages of each of these modalities is debatable, although 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

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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 offers the most detail and potential for standardization but are costly and often require general anesthesia in young children. Regardless of the imaging modality used, it is difficult to justify any clinical study that does not assess the status of glenohumeral development. It is now evident that the internal rotation contracture of the diseased shoulder commonly leads to glenohumeral deformity. Posteriorly directed forces displace the humeral head in the same direction. In growing children, skeletal changes ensue. A false articulation often forms on the posterior aspect of the glenoid that becomes progressively retroverted, leading to a potential array of deformities that have been described as flat, biconcave, and convex (ie, pseudoglenoid). Various classification systems have been proposed.3,7,14,20,21 They have in common the fact that with increasing deformity, there is increasing posterior displacement of the glenohumeral joint from its normally centered and concentric position, and the normal concave shape of the glenoid becomes increasingly convex. In advanced deformities, the humeral head articulates with the posterior aspect of the convex glenoid and becomes increasingly misshapen and retroverted itself18 (Figure 4).

Figure 3

A, Axillary arthrogram of a concentric glenoid fossa in a 10-month-old child with a relatively normal glenohumeral joint. B, Line tracing of panel A outlining the glenoid (GL). Nearly the entire glenoid is unossied cartilage. C, T2-weighted gradient-recalled echo MRI scan of the concentric glenoid in the same patient. Hyaline cartilage appears white (broken arrow) and labral tissue black (solid arrow) on this sequence. The humeral head is round. D, Line tracing of panel C showing the glenoid, scapular center-line, and approximate humeral head center of rotation (white dot). HH = humeral head. (Reproduced with permission from Pearl ML, Edgerton BW, Kon DS, et al: Comparison of arthroscopic ndings with magnetic resonance imaging and arthrography in children with glenohumeral deformities secondary to brachial plexus birth palsy. J Bone Joint Surg Am 2003;85:890-898.)

Surgical Management
A detailed review of the indications and techniques for microscopic surgical 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; apply only to patients in the first year
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of life, after which these options are thought not to be effective; and never result in complete neurologic improvement, always leaving residual impairment. Thus, secondary orthopaedic 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 (<3 months) mi-

crosurgical intervention is recommended. The options are limited to various forms of nerve transfer because grafting is not applicable in the absence of a healthy proximal nerve root.22,23 For intraplexus ruptures, return of antigravity biceps strength remains the primary factor in determining the need for brachial plexus exploration and nerve reconstruction. Controversy persists regarding the need for

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Figure 4

T2-weighted gradient-recalled echo MRI scan of the child in Figures 1 and 3, demonstrating pseudoglenoid conguration. The glenoid contour and scapular center line are enhanced with line tracing. (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 and glenohumeral deformity secondary to brachial plexus birth palsy. J Bone Joint Surg Am 2006;86:564-574.)

finitively claim that microsurgery in combination with secondary orthopaedic procedures results in better outcomes than secondary orthopaedic procedures alone, although much of current practice is predicated on this belief. Smith et al24 presented long-term follow-up of 22 children who had no brachial plexus surgery and for whom biceps recovery was delayed until age 3 to 6 months. These children demonstrated comparable function to that reported for children who had microsurgical repair or grafting to the brachial plexus. Adding to the confusion is the wide array of secondary procedures that have been associated in published accounts with clinical success.

Secondary Interventions Internal Rotation Contracture 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 undertaken at any time because it will not recover spontaneously, although a period 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) anterior capsular release, Z-plasty lengthening of the subscapularis tendon with or without transfer of muscles for external rotation;28 (2) pectoralis major release with transfer of the latissimus and teres major as advocated by Hoffer and colleagues;29,30 (3) subscapularis slide with and without a latissimus transfer as originally described by Carlioz and Brahimi31 and recommended by Gilbert et al;32 and (4) arthroscopic release of the internal rotation contracture

and timing of microsurgical intervention, with recommendations ranging from age 3 to 9 months.24,25 There is no doubt that with increasing delay in return of biceps function less spontaneous recovery will occur. Waters26 has shown that children who do not develop this ability by age 5 months do not do as well under conservative management as those who receive microsurgical nerve intervention (grafting or neurotization). Efforts continue to refine surgical indications, with some centers recommending a more comprehensive evaluation that includes muscles other than the biceps to predict recovery.27 Discerning which neurologic lesions will improve equally well with late secondary orthopaedic intervention and no early microsurgery, as opposed to early microsurgery followed later by secondary procedures, is difficult. No study to date can de-

with or without latissimus transfer.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 prevailing recommendation is an external rotational osteotomy of the humerus, rotating the arm into a more functional position of external rotation.35 How can these seemingly disparate approaches be reconciled? One way is to recognize that the internal rotation contracture results from the loss of the normal balance between external rotation and internal rotation, principally because of infraspinatus weakness. Different types of procedures that restore muscle balance and stability by reducing internal rotation strength and/or augmenting external rotation strength may demonstrate effectiveness. Similarly, a bony procedure that increases external rotation will improve function. It is also likely that commonly used clinical scoring systems, most notably the Mallet, are too crude to distinguish the outcomes among these approaches.

Formal Anterior Approach

The first surgical releases described in the early 20th century by Fairbank36 and later modified by Sever37 used a traditional anterior deltopectoral approach. To reduce the recurrence rate of the internal rotation contracture and add external rotation 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 release of the upper half of the pectoralis major and the entire subscapularis and anterior capsule, along with

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Figure 5

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 the latissimus tendon and reinsertion of the pectoralis into the subscapularis tendon. (Reproduced with permission from Zancolli 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.)

rerouting (transfer) of the latissimus dorsi and teres major tendons to the pectoralis major stump. Gains in external rotation deteriorated over time, and five patients had significant degenerative changes of the glenohumeral joint. Among the concerns regarding the anterior approach were that it resulted in poor cosmesis and potentially led to anterior dislocation of the glenohumeral joint and/or functionally significant external rotation contractures. Several authors have modified this approach with an aim to address some of these issues. Zancolli and Zancolli38,39 have long advocated 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 securing it to the remaining latissimus
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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 results have been reported with this technique, with an increase of 50 average 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 subluxation surgically when the joints surfaces are already deformed. Recent reports by other authors who use an anterior approach and who warn against releasing the anterior capsule have acknowledged that despite their preoperative intentions, at surgery it was not possible to restore external rotation and reduce the glenohumeral joint without releasing the capsule.40,41 In the series of van der Sluijs et al,40 15 of 19 patients 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 contracted anterior soft tissues, these authors postulate that in many cases excessive retroversion of the humerus obligates an external rotation contracture 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 Modication of the LEpiscopo Procedure

A common surgical approach employed 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 major muscles to the posterior rotator cuff29,30 (Figure 6). Hoffer et al29 first reported on this technique in 11 patients, who achieved an average gain

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Figure 6

Illustrations of partial pectoralis major release (A) and transfer of combined latissimus dorsi and teres major tendons to the 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 7

weakness in the absence of a severe, firmly fixed internal rotation contracture and glenohumeral deformity.

Release of the Subscapularis Origin

Gilbert et al32 have long advocated an approach originated by Carlioz and Brahimi31 in which the subscapularis origin is released and the muscle reflected distally (Figure 7, A). In a report on 65 patients followed more than 5 years after this procedure, Gilbert et al32 noted an average 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 earlier release, these authors recommended transfer of the latissimus dorsi tendon to the posterolateral rotator cuff as well (Figure 7, B). Among the stated principles of this surgical approach is that it avoids releasing the anterior capsule and only

Illustrations demonstrating subscapularis release (A) and transfer of the isolated 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.

of 64 of abduction and 45 of external rotation at a minimum 2-year follow-up. A subsequent report 20 years later presented similar success in another eight children.30 A contention of these authors and others advocating 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 contracture. However, recent reports from different centers that used this surgical approach have shown that despite improvements in shoulder function, this procedure did not improve the glenohumeral deformity present in many of the patients.9,42 It is likely that this surgical approach is most effective for external rotation

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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 external rotation strength in favor of the weak external rotators. In one series, however, there was inadequate release of the capsular contracture in 5 of 25 children, requiring an anterior approach at the same surgical setting.6 A recent long-term follow-up analysis noted that many of the functional 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

Figure 8

Figure 9

Arthroscopic Subscapularis Tenotomy and Capsular Release

In 2003, Pearl33 first reported on arthroscopic release with promising early results (Figure 8). A subsequent report on 33 children confirmed the utility of this procedure.10 The surgical protocol used in these studies followed in part the recommendations of Gilbert et al32 in that young children (3 years) receive arthroscopic release only and older children receive a simultaneous latissimus dorsi tendon transfer. Nineteen children in this series received a release only; 14 had a release combined with a latissimus dorsi transfer. At a minimum 2-year follow-up, the mean passive external rotation increased by 67 (P < 0.005) in the 15 children with a successful arthroscopic release (Figure 9) and by 81 (P < 0.005) in those treated with a primary latissimus dorsi transfer. Four of the 19 younger children failed to improve sufficiently and were successfully treated with subsequent latissimus dorsi transfer performed for either failure to maintain sufficient external rotation strength postrelease or for recurrence of the contracture. All 14 older children (age >3
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Arthroscopic view of a right shoulder from a posterior portal demonstrating the level of subscapularis tenotomy with an electrocautery device (arrow). BT = biceps tendon, HH = humeral head. (Reproduced with permission from Pearl ML: Arthroscopic release of shoulder contracture secondary to birth palsy: An early report on ndings and surgical technique. Arthroscopy 2003;19:577-582.)

Active external rotation 2 years after arthroscopic release in the same child as in Figures 1, 3, and 4. (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 and glenohumeral deformity secondary to brachial plexus birth palsy. J Bone Joint Surg Am 2006;88:564-574.)

years) treated with simultaneous release and latissimus transfer maintained gains in external rotation. At follow-up, the four younger children who required delayed latissimus transfer had a mean passive external rotation of 78 and showed no ill effects of delayed transfer. This differential approach to managing children younger than age 3 years with an isolated arthroscopic release remains the authors preferred method of managing these contractures. In the report by Pearl,33 arthroscopic release not only improved external rotation but also demonstrated remarkable remodeling of glenohumeral deformity when present before surgery. Follow-up MRI was available for 15 of 18 children with advanced pseudoglenoid deformities at the time of release or transfer. All but the three most severe deformities (12 of 15) showed normalization of the glenohumeral joint on follow-up MRI scan, as evidenced by increased sphericity of the humeral head, restoration of the glenoid 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 release as long as external rotation is restored and preserved at follow-up. Pedowitz et al34 showed some improvement of glenohumeral alignment 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 current protocol of arthroscopic release (Figure 10). Hui and Torode20 also reported improved glenoid retroversion for 23 children at an average follow-up of 43 months after open anterior release. Most recently, Waters and Bae43 reported on 23 children with 83% showing remodeling of deformity after open soft-tissue procedures that included open glenohumeral joint reduction (ie, capsulorrhaphy). This was in contrast to their

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Figure 10

Figure 11

A, Preoperative T2-weighted gradient-recalled echo MRI scan of a 4.7-yearold patient revealing mild pseudoglenoid. B, T2-weighted gradient-recalled echo MRI scan of the same patient 2 years after arthroscopic release and latissimus dorsi transfer, demonstrating remodeling to a concentric joint with a round humeral head well centered on the glenohumeral joint. (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 and glenohumeral deformity secondary to brachial plexus birth palsy. J Bone Joint Surg Am 2006;86:564-574.)

T2-weighted gradient-recalled echo MRI scan taken 3.5 years postoperatively of a child who had a pseudoglenoid prior to extraarticular subscapularis slide at age 2 years. The scan shows a round humeral head well centered on a concave glenoid.

earlier report on patients treated similarly 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 external rotation, either by sacrificing internal rotator strength or augmenting external rotator power, will inevitably weaken internal rotation or diminish internal rotation range. A loss of internal rotation was observed with the arthroscopic approach, as well.10 As reported, The ability to reach up the back was not measured preoperatively, but it was clearly restricted at the time of followup, with the children only able to reach between the sacrum and L5 on the average.10 Earlier reports in the literature describing results from other techniques have not addressed the resultant 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 improvement from a pre-operative 1 to a postoperative 2 (the ability to reach S1 postoperatively). From these two reports, it appears that both surgical approaches (arthroscopic and open capsulorraphy) result in the same amount of internal rotation.10,43 It is not clear why this was observed as a decrease in function in the arthroscopic study and an increase following capsulorraphy, but the difficulties in examining motor function in young children may play a role. One may conclude from the literature on the various techniques of contracture release and tendon transfer, as just discussed, that procedures that do not release the anterior capsule will less consistently restore external rotation and improve glenohumeral deformity

than those that target the joint capsule. Accordingly, historic concerns regarding release of the subscapularis and the anterior capsule must be tempered by the evidence that not doing so will not release all contractures and will leave behind many posteriorly displaced, deformed glenohumeral joints without the possibility of remodeling. The arthroscopic release allows for select attention to the subscapularis and anterior capsule in contrast to the earlier open procedures that required release of multiple superficial structures as well.

Rotational Osteotomy
For older children or those with advanced glenohumeral deformity, the prevailing recommendation has been to avoid soft-tissue procedures at the joint in favor of rotational osteotomy of the humerus. The utility of this procedure was confirmed in a recent report by Waters and Bae.35 These authors reported on 27 pa-

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tients who underwent rotational osteotomy with plate fixation and achieved an average improvement of 64 in external rotation and improved 5 points on a modified Mallet scale. No clear guidelines exist, however, as to what age or degree of deformity should dictate this form of surgical treatment. As it is now evident that many children younger than age 6 years are capable of substantial 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 remodeling. At present, the surgeon must make a judgment call regarding the growth potential of the child, the severity 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).

Figure 12

A, Preoperative T2-weighted gradient-recalled echo MRI scan of an 11-yearold boy with a functionally disabling internal rotation contracture. His age and extensive glenohumeral deformity made signicant skeletal remodeling and improvement with soft-tissue procedures unlikely. B, Postoperative anteroposterior radiograph demonstrating that rotating the humerus 60 into external rotation can dramatically improve appearance and function.

Other Problems Poor Active Elevation

Weakness of elevation is functionally limiting when active elevation is <90. Affected children have much better passive than active ROM. Some reports of anterior capsular release and muscle transfer to provide or increase external rotation claim significant improvement in abduction with these procedures.29,30,39,44 However, we have observed only modest improvements in active elevation with any of the aforementioned procedures and believe that much of the improvement noted in other studies reflects the difficulties in measuring motion in young children as well as the apparent increased reach resulting from procedures that improve external rotation. Among the specific procedures designated to improve active elevation are
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upper trapezius transfer, in which that muscle is detached from the acromion and inserted distally on the humerus, and bipolar transfer of the latissimus dorsi and pectoralis major to replace the deltoid. No recent clinical series have demonstrated success with any of these procedures. Currently, shoulder elevation in brachial plexus birth palsy is largely an unsolved problem, and aggressive surgical intervention is likely to be disappointing. However, for a child with limited elevation and a significant internal rotation contracture, improving the range of external rotation can markedly improve the childs ability to reach, by allowing the hand to be placed in a higher position (Figure 13).

Scapular Dyskinesia
Asymmetric and apparently abnormal movement of the scapula is a frequent cause of concern to parents. It is most often associated with an internal rotation contracture but will

accompany stiffness or limited excursion of the glenohumeral joint in any direction. Zancolli and colleagues38,39 refer to the scapular elevation sign and have noted that it accompanies internal, external, and abduction contractures. The sign, historically called the Putti sign, is seen when the superior angle of the scapula bulges into the trapezius on forced external rotation of the arm in the patient with an internal rotation contracture. Conversely, the so-called reverse Putti sign demonstrates aberrant scapular motion with forced internal rotation in the presence of an external rotation contracture. Surgical release of the contractures improves scapular motion, but incompletely. For external rotation and abduction contractures involving the supraspinatus and the posterior rotator cuff, surgical release must include these important motors of the shoulder. Zancolli and colleagues38,39 have reported such a surgical technique but did not report on clinical results.

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Figure 13

Postoperative active elevation of 140 (20 gain from the preoperative state) 2 years after arthroscopic release in the same child as shown in Figures 1, 3, 4, and 9. Note the improved ability to reach, with improved external rotation.

Scapular dyskinesia may exist even when the internal rotation contracture is not severe enough to compel surgical treatment. Although some weakness of the rhomboid and serratus 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 frozen shoulder, the ROM in these neurologically damaged shoulders is globally restricted. Tightness in the superior structures such as the supraspinatus will result in an abduction contracture of the humerus relative to the scapula. In severe cases, as

the child lowers the arm to the side, the scapula will elevate. With movement, as the child tries to position the hand in space,38,39 the scapula will be carried along in an asymmetric manner. This abnormal scapular movement does not represent a motor problem of the scapular muscles, but is secondary to changes at the glenohumeral joint in the most extreme 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 glenohumeral deformity. They offer a surgical protocol aimed at correcting this root cause. Although this approach does focus interest on a perhaps neglected concern of the parent or guardian (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 scapulae) than on the muscles that externally rotate the shoulder. It is incongruous to attribute an internal (medial) rotation contracture to aberrant scapular motion when the more proximate cause is readily evident.

following release of internal rotation contractures. Surgical intervention is indicated when functional limitations in personal care are severe and the child has great difficulty reaching the midline. Zancolli and Zancolli39 described surgical release and lengthening of the posterior-superior rotator cuff for this contracture. Few others have experience with this approach, 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 appropriately selected children both cosmetically and functionally in terms of their ability to reach the midline to 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 microsurgical intervention has become clearer and the techniques refined, complete restoration of neuromuscular function remains elusive. For motor weakness that follows the neurologic injury, internal rotation contractures most commonly occur as a result of external rotation weakness. This leads to deformity of the glenohumeral joint (ie, glenohumeral dysplasia, posterior humeral head subluxation). As a result, secondary orthopaedic evaluation and corrective 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 glenohumeral joint. A variety of surgical approaches to restore balance between external rotation and internal rotation power has demonstrated

External Rotation Contractures

External rotation contractures present a smaller subset of the problems encountered from birth palsy than internal rotation contractures, with 14% reported by Zancolli and Zancolli.38 This contracture does not result in posterior displacement of the glenohumeral joint or in significant deformity, but it can be functionally disabling. External rotation contracture may also occur iatrogenically

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Michael L. Pearl, MD

clinical success. Currently, clinical studies do not definitively favor one approach, but existing evidence supports early correction of the internal rotation contracture and reduction of the glenohumeral joint by open or arthroscopic means, even if it requires release of the anterior capsule. Surgeons who have experience with both open and arthroscopic methods usually find that the arthroscopicguided release allows for equal if not greater contracture release, with reduced morbidity. Regardless of the method used for anterior capsule release, most surgeons who treat brachial plexus birth palsy favor supplementing the release with a transfer of the latissimus dorsi tendon, whether in all children or in children older than age 3 years. Consideration of any of the procedures 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. Similarly, an elbow that cannot extend because of a persistent flexion contracture or an absent triceps may impede a childs reach to a far greater extent than limitation of shoulder motion.

contracture. Clin Rehabil 2000;14:523526. 3. Hoeksma AF, Ter Steeg AM, Dijkstra P, Nelissen RG, Beelen A, de Jong BA: Shoulder contracture and osseous deformity in obstetrical brachial plexus injuries. J Bone Joint Surg Am 2003;85: 316-322. Bisinella GL, Birch R: Obstetric brachial plexus lesions: A study of 74 children registered with the British Paediatric Surveillance Unit (March 1998-March 1999). J Hand Surg [Br] 2003;28:40-45. van der Sluijs JA, van Ouwerkerk WJ, de Gast A, Wuisman PI, Nollet F, Manoliu RA: Deformities of the shoulder in infants younger than 12 months with an obstetric lesion of the brachial plexus. J Bone Joint Surg Br 2001;83:551-555. Pearl ML, Edgerton BW: Glenoid deformity secondary to brachial plexus birth palsy. J Bone Joint Surg Am 1998; 80:659-667. Kozin SH: Correlation between external rotation of the glenohumeral joint and deformity after brachial plexus birth palsy. J Pediatr Orthop 2004;24:189193. Pagnotta A, Haerle M, Gilbert A: Longterm results on abduction and external rotation of the shoulder after latissimus dorsi transfer for sequelae of obstetric palsy. Clin Orthop Relat Res 2004;426: 199-205. Waters PM, Bae DS: Effect of tendon transfers and extra-articular soft-tissue balancing on glenohumeral development in brachial plexus birth palsy. J Bone Joint Surg Am 2005;87:320-325. Pearl ML, Edgerton BW, Kazimiroff PA, Burchette RJ, Wong K: Arthroscopic release and latissimus dorsi transfer for shoulder internal rotation contractures and glenohumeral deformity secondary to brachial plexus birth palsy. J Bone Joint Surg Am 2006;88:564-574. Curtis C, Stephens D, Clarke HM, Andrews D: The active movement scale: An evaluative tool for infants with obstetrical brachial plexus palsy. J Hand Surg [Am] 2002;27:470-478. Bae DS, Waters PM, Zurakowski D: Reliability of three classification systems measuring active motion in brachial plexus birth palsy. J Bone Joint Surg Am 2003;85:1733-1738. Saifuddin A, Heffernan G, Birch R: Ultrasound diagnosis of shoulder congruity in chronic obstetric brachial plexus palsy. J Bone Joint Surg Br 2002; 84:100-103. Moukoko D, Ezaki M, Wilkes D, Carter P: Posterior shoulder dislocation in infants with neonatal brachial plexus palsy. J Bone Joint Surg Am 2004;86:

787-793. 15. Pyhi TH, Nietosvaara YA, Remes VM, Kirjavainen MO, Peltonen JI, Lamminen AE: MRI of rotator cuff muscle atrophy in relation to glenohumeral joint incongruence in brachial plexus birth injury. Pediatr Radiol 2005;35:402-409. Pearl ML, Edgerton BW, Kon DS, et al: Comparison of arthroscopic findings with magnetic resonance imaging and arthrography in children with glenohumeral deformities secondary to brachial plexus birth palsy. J Bone Joint Surg Am 2003;85:890-898. van der Sluijs JA, van Ouwerkerk WJ, Manoliu RA, Wuisman PI: Secondary deformities of the shoulder in infants with an obstetrical brachial plexus lesions considered for neurosurgical treatment. Neurosurg Focus 2004;16:E9. van der Sluijs JA, van Ouwerkerk WJ, de Gast A, Wuisman P, Nollet F, Manoliu RA: Retroversion of the humeral head in children with an obstetric brachial plexus lesion. J Bone Joint Surg Br 2002; 84:583-587. van der Sluijs JA, van der Meij M, Verbeke J, Manoliu RA, Wuisman PI: Measuring secondary deformities of the shoulder in children with obstetric brachial plexus lesion: Reliability of three methods. J Pediatr Orthop B 2003; 12:211-214. Hui JH, Torode IP: Changing glenoid version after open reduction of shoulders in children with obstetric brachial plexus palsy. J Pediatr Orthop 2003;23:109113. Kon DS, Darakjian AB, Pearl ML, Kosco AE: Glenohumeral deformity in children with internal rotation contractures secondary to brachial plexus birth palsy: Intraoperative arthrographic classification. Radiology 2004;231:791795.

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