Brachial Plexus Birth Palsy: An Overview of Early Treatment Considerations
Brachial Plexus Birth Palsy: An Overview of Early Treatment Considerations
Brachial Plexus Birth Palsy: An Overview of Early Treatment Considerations
David E. Ruchelsman, M.D., Sarah Pettrone, M.D., Andrew E. Price, M.D., and John A.I.
Grossman, M.D., F.A.C.S.
Abstract same period, both Erb and Klumpke further elucidated the
Since the description by Smellie in 1764, in a French mid- anatomic details of different clinical presentations. During
wifery text, that first suggested an obstetric origin for upper the early 20th Century, results following neurosurgical in-
limb birth palsy, great strides have been made in both diag- tervention were dismal. This lack of interest in early nerve
nosis and early and late treatment. This report presents an repair remained unchanged for over half a century until
overview of selected aspects of this complex and extensive 1984 when Gilbert demonstrated that using microsurgi-
subject. Early treatment options are reviewed in the context cal technique coupled with intraoperative neurophysi-
of the present controversies regarding the natural history and ologic monitoring could, in fact, dramatically change
the indications for and timing of microsurgical intervention the outcome in properly selected infants.2 The role and
in infants with brachial plexus birth injuries. timing of microsurgical repair and reconstruction remains
the most debated issues among contemporary surgeons
A
fter the initial description of a brachial plexus birth specializing in the care of these children.
palsy by Smellie in 1764 (Fig. 1),1 over a century
passed before Duchenne confirmed that this com- Anatomy
plex insult likely resulted from birth trauma and was not The brachial plexus is composed of the ventral motor nerve
“congenital” in origin. He described four cases of C5-C6 roots from C5 to T1 (Fig. 2). Twenty-two percent of anatomic
injury with the resultant shoulder paralysis. During the specimens receive contributions from C4 (prefixed) and 1%
contributions from T2 (postfixed). The roots then combine
David Ruchelsman, M.D., is an Administrative Chief Resident,
to form trunks, divisions, cords, and branches. The C5 and
Department of Orthopaedic Surgery, NYU Hospital for Joint
C6 nerve roots form the upper trunk, C7 alone forms the
Diseases. Sarah Pettrone, M.D., was an Administrative Chief
Resident, Department of Orthopaedic Surgery, NYU Hospital for middle trunk, and the C8 and T1 nerve roots form the lower
Joint Disease and is currently in private practice with Common- trunk. Each trunk then has an anterior and posterior divi-
wealth Orthopaedics in Reston and Leesburg, Virginia. Andrew E. sion. The posterior divisions of all three trunks combine to
Price, M.D., is Clinical Associate Professor, New York University form the posterior cord. Each cord is named by its anatomic
School of Medicine, and an Attending in the Division of Pediatric relationship to the axillary artery. The terminal branches of
Orthopaedics, Department of Orthopaedic Surgery, NYU Hospital the posterior cord are the radial and axillary nerves. The
for Joint Diseases. John A.I. Grossman, M.D., is Clinical Assistant anterior divisions of the upper and middle trunks form the
Professor of Orthopedic Surgery, New York University School of lateral cord, whose terminal branch is the musculocutaneous
Medicine and an Attending in the Division of Hand and Wrist Sur- nerve. The anterior division of the lower trunk and a branch
gery, Department of Orthopaedic Surgery, NYU Hospital for Joint
from the lateral cord forms the medial cord, whose terminal
Diseases, NYU Langone Medical Center, New York, New York.
branches are the median and ulnar nerves.
He is also in private practice in Miami and Director of the Brachial
Plexus Program at Miami Children’s Hospital, Miami, Florida. It is important to note that the brachial plexus supplies
Correspondence: David E. Ruchelsman, M.D., Department of every muscle of the upper extremity except the trapezius.
Orthopaedic Surgery, NYU Hospital for Joint Diseases, 301 East Furthermore, the upper trunk provides primarily shoulder
17th Street, Suite 1402, New York, New York 10003; david.ruchels- function, whereas the lower trunk provides primarily hand
[email protected]. function.
Ruchelsman DE, Pettrone S, Price AE, Grossman JAI. Brachial plexus birth palsy: an overview of early treatment considerations. Bull NYU Hosp Jt Dis.
2009;67(1):83-9.
84 Bulletin of the NYU Hospital for Joint Diseases 2009;67(1):83-9
Figure 2 Schematic of brachial plexus anatomy. (Reproduced from Waters PM. Obstetric brachial plexus injuries: evaluation and man-
agement. © 1997 American Academy of Orthopaedic Surgeons. Reprinted from the Journal of the American Academy of Orthopaedic
Surgeons, Volume 5 (4), pp. 205-214. With permission.)
Bulletin of the NYU Hospital for Joint Diseases 2009;67(1):83-9 85
Classification
Perhaps, for us, the most useful classification of brachial
plexus birth lesions is a clinico-anatomic one and will form
the basis of the remainder of this discussion. C5-C6 le-
Figure 3 Preoperative MRI axial image demonstrating neuroma sions involve paralysis of primarily upper trunk innervated
of the left brachial plexus. Note the associated glenohumeral muscles. Clinically, these infants present with weakness of
pathology with glenoid dysplasia and posterior subluxation of the
shoulder elevation and external rotation, absent biceps, and
humeral head.
the classic shoulder internal rotation and elbow extension
hand.” The involved upper extremity will have decreased posture. C5-C6-C7 lesions also show weakness of both tri-
spontaneous movement and the Moro reflex will be absent. ceps and wrist extension, and sometimes thumb weakness.
If the lower trunk is involved, the child may have an absent Global palsies present at birth with a generally flail, insensate
grasp reflex, the hand may be held in an intrinsic minus limb, and often upper eyelid ptosis.
position, or the involved extremity may have a flaccid pa- We present here a focused review of the contemporary
ralysis. Respiratory distress may also be seen when there early treatment options for these infants. Delineation of the
is an associated injury to the phrenic nerve, although this precise natural history of OBPP remains elusive because of
is rare. A chest radiograph or ultrasound will confirm this the heterogeneous cohorts of patients, methodological flaws,
clinical scenario. The child may also present with a Horner’s and inability to ensure long-term follow-up in these children
syndrome if there has been injury to the sympathetic chain. in early retrospective series. These limitations explain the
Anatomically the sympathetic chain lies in close proximity to intense controversies that continue to surround the critical
the lowest roots of the brachial plexus, making it susceptible questions regarding the specific indications and optimal tim-
to injury. The clinical triad of Horner’s syndrome consists ing of “early” microsurgical intervention in these children.
of miosis, ptosis, and anhydrosis. The upper eyelid ptosis
is most readily detected. Natural History Following Nonoperative
The differential diagnosis of OBPP can include infec- Treatment of Brachial Plexus Birth Injuries
tions, such as septic arthritis of the shoulder and acute A critical analysis of recent published data4,5,7,11,14-19 sug-
osteomyelitis. Fracture of the humerus or clavicle will also gests that, indeed, the majority of infants will demonstrate
present with a pseudoparalysis of the shoulder. Although spontaneous ongoing motor recovery with observation alone.
rare, congenital malformations of the plexus can also occur. A study from the British pediatric surveillance unit found
Spinal cord injury, cerebral palsy, or other central nervous that 53% of children with OBPP spontaneously recovered
system (CNS) lesions can also be initially present with to normal or nearly normal levels, while an additional 39%
hypotonia of the involved upper extremity. regained “good” function of the upper limb.19 It is well
The utility of magnetic resonance imaging (MRI) (Fig. accepted that classic Erb’s palsy has the best prognosis,
3) in the evaluation of a child with OBPP is useful and has with up to 90% reported rates of spontaneous recovery.20
replaced more invasive CT (computed tomography) my- C7 involvement, however, is associated with an 80% risk
elography. In a young child, this test requires sedation or of poor recovery in upper plexus palsies. Lower plexus
formal anesthesia, which is not without risk in this patient involvement has the poorest prognosis, with some studies
population. While myelographic or MRI evidence of pseu- showing that less than 10% will recover any useful function
domeningoceles is associated with nerve root avulsions, the of the hand.4 Due to its poor prognosis, many centers use
testing characteristics of these modalities in infants remain pan-plexus lesions as an indication for early (i.e., 3 months
suboptimal. CT myelogram has only 69% sensitivity, 89% of age) surgical intervention.
specificity, and a positive predictive value of 50% for de- Gilbert and Tassin’s2 landmark series demonstrated that
tecting nerve root avulsions. The negative predictive value the absence of biceps anti-gravity function by 3 months of
of CT myelogram is 93%.11 The MRI finding of a pseudo- age portends a poor prognosis with regard to shoulder func-
meningocele has a low sensitivity (approximately 50%), tion. A single-institution longitudinal study15 of 91 infants
but a high specificity (approaching 100%) in the diagnosis treated nonoperatively with only physical and occupational
of nerve root avulsion.12 MRI information is valuable for therapy over a 7-year period highlighted the variable subco-
preoperative planning; intraoperatively, this information horts comprising this complex patient population and their
is supplemented with neurophysiology and histopathology associated unique clinical courses with observation alone.
86 Bulletin of the NYU Hospital for Joint Diseases 2009;67(1):83-9
Table 1 Medical Research Council Muscle Grading Similarly, in a prospective study of 66 patients designed
System to compare the natural history of nonoperative manage-
Observation Grade
ment of OBPP with that of microsurgical repair in patients
who demonstrated no biceps function at six months of age,
No contraction M0
Trace contraction M1 Waters14 found that children without biceps recovery after
Active movement-gravity eliminated M2 3 months were all Narakas groups 3 and 4 (i.e., more severe
Active movement against gravity M3 neurological involvement with persistently weak digital
Active movement against gravity and resistance M4 extensors or flail arm). The later the return of biceps func-
Normal strength M5 tion the poorer the ultimate shoulder function. The scores
for every aspect of the Mallet shoulder function (Fig. 4)
Specifically, 63 children with an upper or upper-middle decreased with delayed biceps return. In this series, no child
plexus injury (Group 2) recovered good-to-excellent shoul- with biceps recovery after 3 months had full spontaneous
der and hand function at a minimum of two-year follow-up. recovery of shoulder function.
Additionally, serial evaluations documented British Medical In a review of 28 patients with absent biceps function
Research Council M4 or better motor strength in the biceps at 3 months, Smith and colleagues reported that no patient
and deltoids (Table 1). In contrast, 12 infants with global recovered normal shoulder function, and only 12 patients
palsies (Group 1) demonstrated only M0 to M1 in these regained good shoulder function.6 Specifically, shoulder
critical muscles at 6 months of age. Sixteen patients with a function decreased with increasing delay in biceps recovery.
similar clinical presentation to Group 2 (Group 3) demon- All patients with C5 and C6 lesions regained biceps function
strated minimal spontaneous recovery of biceps and deltoid by 6 months. Conversely, no patient with Horner’s syndrome
function by 6 months of age. These observations suggest at birth regained biceps function by 6 months.
that children with rapidly progressive motor recovery during These recent data5,6,11,14-23 allow for several conclusions
the first 3 to 4 months of life are manifesting resolution of that impact the current algorithm of treatment in these chil-
a neuropraxic injury, while infants with upper and upper- dren:
middle plexus injuries, who fail to show motor improvement 1. The majority of upper brachial plexus birth injuries
between 3 to 6 months of age (i.e., Group 3), have likely are transient;
sustained a neurotemetic or dense axonotemetic injury, and 2. Global palsies have a poor prognosis with nonopera-
should be managed in a similar fashion to infants with global tive treatment;
palsies. Furthermore, all children with minimal spontaneous 3. Failure to recover anti-gravity biceps function by 3
recovery of deltoid and biceps function by 6 months of age to 6 months of age is a poor prognostic sign; and
were found to have only poor-to-fair shoulder function and 4. Infants with C5-C6 or C5-C6-C7 injuries may
fair-to-satisfactory hand function on the Gilbert Shoulder and sometimes continue to demonstrate spontaneous
Gilbert-Raimondi Hand scales, respectively (Tables 2 and improvement between 3 and 6 months of age; thus,
3). It is thus crucial to perform thorough serial examinations precluding the need for early surgery.
in order to identify this subcohort of children who should Therefore, the true challenge to the treating physician is the
be offered early microsurgical intervention. correct identification of the subcohort of infants who will
either plateau or fail to show any neurological improvement.
This highlights the crucial need for careful serial, complete
neurological examinations.
Table 3 Gilbert/Raimondi Classification of Impairment of the Hand in Patients with Obstetric Palsy
Grade (Function) Criteria
0 (None) Complete paralysis or slight finger flexion of no use, useless thumb—no pinch, some or no sensation.
1 (Poor) Limited active flexion of fingers; no extension of wrist or fingers; possibility of thumb lateral pinch.
2 (Fair) Active extension of wrist with passive flexion of fingers (tenodesis)—Passive lateral pinch of thumb
(pronation).
3 (Satisfactory) Active complete flexion of wrists and fingers—mobile thumb with partial abduction—opposition intrinsic
balance—no active supination; good possibilities for palliative surgery.
4 (Good) Active complete flexion of wrist and fingers; active wrist extension—weak or absent finger extensor; good
thumb opposition with active ulnar intrinsics; partial prosupination.
5 (Excellent) Hand IV with finger extension and almost complete prosupination.
was used to predict recovery at 12 months, the proportion ers21 reported promising results in 54 children following
of patients whose recovery was incorrectly predicted was isolated spinal accessory to suprascapular nerve transfers,
12.8%, implying that 1 in 8 patients would have had surgery performed as a primary or secondary procedure at a mean
unnecessarily or would not have had surgery when required. of 21 months of age in children without return of active
When elbow flexion was combined with elbow, wrist, thumb, shoulder external rotation. A recent review of our experience
and finger extension, recovery was incorrectly predicted in suggests that direct spinal accessory to suprascapular nerve
only 5.2% of cases.7 Similarly, Clarke and Curtis11 observed transfer provided similar clinical and functional outcomes
that 10% to 15% of infants without biceps contraction at when performed prior to or after 9 months of age.30 While
3 months would still spontaneously recover by 9 months. these studies are retrospective and without a control group in
The senior investigator’s (JAIG) observations support this which established musculoskeletal procedures (contracture
as well. release, tendon transfer, humeral osteotomy) are performed,
Persistent deficits in active wrist extension at 4 to 5 the results achieved following late nerve reconstruction ap-
months with recovery of hand function is highly suggestive pear to warrant this approach in experienced centers.
of a poor outcome, especially regarding the shoulder without
surgical intervention. Microsurgical Options
The clinical approach for children older than 8 to 12 With advancement in microsurgical techniques by Narakas,31
months of age with persistent proximal motor weakness Millessi,32 Gilbert and Tassin,2 Kawabata and colleagues,33
and fixed or progressive shoulder deformity has received and others, interest in the microsurgical reconstruction of
little attention. Often, delayed orthopaedic procedures (i.e., brachial plexus injuries grew. An in-depth discussion of cur-
contracture release, tendon transfers, humeral osteotomy) are rent microsurgical techniques and options used for brachial
recommended. Birch and coworkers24 reported promising plexus reconstruction is beyond the scope of this paper, but
results following late grafting into the suprascapular nerve. the spectrum includes neurolysis, neuroma resection with
Subsequent series28,29 suggest that late nerve reconstruction nerve grafting, neurolysis and bypass grafting, and nerve
in properly selected infants with persistent severe shoul- transfers (Fig. 5). Direct repair is rarely performed due to
der sequelae and upper plexus injuries may be beneficial. the extensive nature of these injuries and the inability to
Grossman and colleagues29 reported an improvement of achieve a tension-free repair without interposition grafting.
two grades or more on the modified Gilbert shoulder scale While Laurent and associates34 advocate performance of
in 22 infants who underwent combined plexus and shoulder neurolysis alone in the presence of a neuroma-in-continuity,
reconstruction at a mean age of 16 months. Grossman and which demonstrates greater than 50% maintenance of action
associates28 have also shown that following neurolysis alone potential upon intraoperative stimulation, many surgeons
(n = 3) or neurolysis with bypass grafting (n = 8) at a mean have abandoned this approach.35,36 Capek and colleagues37
age of 13 months, the median increase by the modified reported better long-term results with neuroma resection and
Gilbert shoulder scale was 2 grades and that 6 of 11 cases grafting, as compared to neurolysis alone in the setting of
improved by at least 3 grades. Van Ouwerkerk and cowork- conducting and nonconducting neuromas, despite an initial
88 Bulletin of the NYU Hospital for Joint Diseases 2009;67(1):83-9
Multidisciplinary Approach
A multidisciplinary approach is crucial to optimizing the
outcome of these children. Our model consists of a team
comprised of specialists in child neurology, neurophysiol-
ogy, and occupational therapy who serially evaluate these
infants. The collective combined expertise of these special-
Figure 5 Intraoperative view of the regional anatomy following
surgical approach to the brachial plexus and microsurgical neuroly-
ists is used in collaboration with the treating orthopaedic and
sis. C5, C5 nerve root; P, phrenic nerve; N, neuroma of the upper peripheral nerve and hand surgeons for decision making in
trunk; SS, suprascapular nerve; AD, anterior division of the upper the immediate perioperative period and during long-term
trunk; PD, posterior division of the upper trunk. follow-up. Experienced neuroradiologists, pathologists, and
pediatric anaesthesiologists aswell as pediatric hospitalists
down-grading following resection. To avoid initial down- round out the team.
grading, some experts advocate neurolysis with bypass
grafting over neuroma resection and graft reconstruction. Summary
The intraoperative decision-making process is complex and Infants with brachial plexus birth injuries represent a com-
requires consideration of multiple preoperative and intraop- plex, heterogeneous group of patients. Current controver-
erative findings. Interestingly, despite the long duration and sies surrounding optimal timing and early microsurgical
complexities of these procedures, the complication rate is intervention will continue even as large patient cohorts and
extremely low.38 In an analysis of a single-surgeon experi- long-term follow-up designed to answer these questions
ence of 100 consecutive cases over a 30-month period, there are available. There is no “cookie-cutter” approach avail-
were no major complications (i.e., phrenic nerve injury, able to treat these unique patients; intraoperative decision
pneumothorax, chylothorax, pulmonary edema, or wound making is based on the extent of the injury, preoperative and
infection), and only eight minor complications (intraopera- intraoperative findings, available reconstructive options, and
tive wheezing, n = 5; prolonged hospitalization for poor oral surgeon experience. The learning curve is significant with
intake, n = 2; and bronchiolitis, n = 1).38 regard to both the nonoperative and operative management
Glenohumeral deformity occurs early and is progressive of these children. Serial and thorough clinical examinations
in children who do not demonstrate spontaneous recov- of the infant are paramount to identifying the small segment
ery39,40; its occurrence is secondary to the muscle imbalance of patients who will definitively benefit from early surgical
that develops about the shoulder. At the time of primary intervention. A multidisciplinary approach incorporating
plexus exploration, the glenohumeral joint must be assessed. various health professionals is crucial to optimizing func-
Our indications to perform a concomitant glenohumeral re- tional outcomes in these children.
construction include inability to obtain full passive external
rotation of the shoulder when the upper limb is positioned Acknowledgment
adducted at the patient’s side and the elbow flexed, or if there The authors sincerely thank Ms. Sandy Allen, medical editor,
is joint instability. Reconstructive options for the associated and Mr. Herbert Valencia, R.N.F.A., for their tremendous
internal rotation contracture include capsuloplasty, open help and support in the preparation of this manuscript.
reduction and posterior capsulorrhaphy, pectoralis major
tenotomy, and subscapularis slide. Further, there is data to Disclosure Statement
support consideration of botulinum toxin type A injections None of the authors have a financial or proprietary interest
into the internal rotation muscle groups about the shoulder in the subject matter or materials discussed, including, but
(i.e., pectoralis major and the latissimus dorsi-teres major not limited to, employment, consultancies, stock ownership,
complex) at the time of the primary plexus reconstruction honoraria, and paid expert testimony.
(based on the experience of AP and VAIG). Use of botulinum
toxin in this setting has been shown to reduce the incidence References
and severity of recurrent internal rotation contracture of the 1. Smellie W. Collection of Preternatural Cases and Observa-
shoulder following a subscapularis slide.41 Finally, it must tions in Midwifery (Vol 3). London: Wilson and Durham,
Bulletin of the NYU Hospital for Joint Diseases 2009;67(1):83-9 89