Fig. 36.1 (a) Diagram of the Wassel classification scheme for thumb polydactyly. (b) Clinical example of a Wassel type II thumb polydactyly. (c) Clinical
example of type III thumb polydactyly. (d) Clinical example of type IV thumb polydactyly.
Fig. 36.2 (a) Clinical example of central polydac-
tyly. (b) Anteroposterior radiograph of the same
hand.
Polydactyly
333
36.2 Diagnosis
The diagnosis of polydactyly often will be evident on presenta-
tion. Investigations will di erentiate the skeletal involvement of
the polydactylous digit(s) based on both clinical examination
and standard radiographs. Radiographs will determine the
presence of an articulation of the polydactylous digit, the level
of articulation, and the presence of an additional metacarpal
( Fig. 36.6). During the clinical examination, it is important
to expose all limbs to identify additional polydactylies. Radial
and ulnar polydactylies may be isolated or part of a syndrome;
as such, a thorough examination of the child apart from
the extremities is warranted to rule out other underlying
malformations. Special attention for café au lait spots and short
stature is warranted in radial polydactyly patients due to the
association with Fanconi anemia.
36.3 Nonoperative Management
36.3.1 Radial Polydactyly
Radial polydactyly is rarely managed nonoperatively. In mild
cases of a Wassel type I deformity with a bifid distal phalanx
resulting only in a widened thumb tip and minimal nail abnor-
mality, observation may be reasonable. Otherwise, surgical in-
tervention is recommended for radial polydactyly.
36.3.2 Central Polydactyly
Surgical management is the mainstay for treatment of central
polydactyly. However, if only a small soft-tissue mass is present,
ligation may be an option. For more information, see the Liga-
tion section for ulnar polydactyly.
36.3.3 Ulnar Polydactyly
Observation
Observation is an option for both types A and B ulnar polydac-
tyly; however, the supernumerary digit can cause functional
deficits, particularly with pedunculated digits that can catch on
objects. Observation is likely most appropriate when a nubbin
is present and is asymptomatic.
Ligation
Other nonsurgical options for type B include ligation, usually
with a 4.0 silk suture or a surgical clip. Ligation has traditionally
been the most common form of treatment for pedunculated
type B digits, producing necrosis of the digit and subsequent
autoamputation. Ligation can be done in the nursery shortly
after delivery, but can be performed at a later stage ranging
from 2 to 40 weeks (average 8 weeks of age). Rayan et al
Fig. 36.3 Example of a distal type III polydactyly of the index finger.
Fig. 36.4 A clinical example of type A ulnar polydactyly.
Upper Extremity
334
recommended ligation only for digits with narrow stalks with
suture placement as close to the finger to limit nubbin forma-
tion. Additionally, Mullick et al recommend limiting this treat-
ment to stalks <2 mm with the presence of any bony or
ligamentous attachments as a contraindication to ligation. Close
observation for bleeding and infection and a discussion with
parents about the risk of potential revision procedures are also
important components of this management strategy. This tech-
nique has become controversial due to the concern of associ-
ated complications (see Complications section). A recent poll of
pediatricians and hand surgeons in the United Kingdom on
referral to a hand surgeon versus ligation in the neonatal period
demonstrated that the majority of clinicians would refer for
surgical assessment. However, proponents of ligation cite its
low cost and simplicity.
36.4 Operative Treatment
36.4.1 Radial Polydactyly
Treatment of radial polydactyly is typically surgical and can be
initiated at 6 to 9 months of age. The goals of surgery are both
functional and cosmetic. Preoperative surgical splinting has
been found to be valuable for improving angular and rotatory
deformities prior to surgical correction. There are two primary
surgical treatment options: (1) ablation with collateral ligament
reconstruction or (2) the Bilhaut–Cloquet procedure.
Ablation with collateral ligament reconstruction is preferred
for duplications with asymmetry resulting in the presence of a
larger dominant thumb and smaller hypoplastic thumb. This
most commonly includes removal of the radial (hypoplastic)
duplicate, centralization of the flexor and extensor mechanism,
collateral ligament reconstruction, and correctional osteoto-
mies for angular deviation. Removing the radial duplicate when
possible leaves the ulnar collateral ligament intact, which is of
primary importance in thumb stability and pinch ( Fig. 36.7).
In a type IV thumb, partial resection of the metacarpal head
may be necessary for proper alignment of the phalanx on the
metacarpal head. Therapy following surgery usually includes
scar management and nighttime splinting for a period of 3 to 6
months. In cases where significant reconstruction of the radial
collateral ligament is required, a full-time thumb spica splint
may be worn for 2 to 3 months for additional stability. It should
be mentioned ablation alone is not recommended for radial
polydactyly treatment as it uniformly leads to joint instability.
The Bilhaut–Cloquet procedure was originally described in
1889. It involved the excision of the central portion of a type I
thumb duplication with preservation of the ulnar aspect of the
ulnar most portion and the radial aspect of the radial most por-
tion of the duplicated distal phalanges. The idea was to allow a
normal-size digit to be created from the two abnormal portions
while maintaining the collateral ligaments for stability. It was
initially used for type I and II deformities but expanded to types
Fig. 36.5 A clinical example of ulnar type B
polydactyly.
Fig. 36.6 Anteroposterior radiograph of type A ulnar polydactyly.
Polydactyly
335
III and IV. The Bilhaut–Cloquet procedure is technically di cult
requiring exact alignment of the nail, bone, and grow th plate
( Fig. 36.8). It may be recommended when the duplicated dig-
its are similar in size. In an e ort to avoid nail grooving and
growth plate problems associated with the Bilhaut–Cloquet
procedure, some surgeons recommend a modification in which
the lateral components of the radial digit are maintained for
digital width, but the entire nail is taken from the ulnar digit.
36.4.2 Central Polydactyly
Surgical care is usually initiated between 6 and 12 months of
age ( Fig. 36.9). The goal is to begin surgical management
before joint angulation and contractures occur. Despite an early
start on surgical management, patients usually require multiple
operations to obtain a cosmetically pleasing and functional
hand. Because central polydactyly is so often seen in combina-
tion with syndactyly, radiographs are necessary to define the
underlying bony anatomy and ideal surgical management.
In type II polydactyly, each phalanx has its own epiphysis. As
the duplicated digit grows, it will displace the normal digit.
Simple ablation is not appropriate and collateral ligament
reconstruction is necessary. As the deformity worsens, correc-
tive osteotomies and joint arthrodesis may be necessary. Tendi-
nous attachments to both the digit to be maintained and that to
be removed should be examined at the time of surgery. The
most appropriate tendon should be retained. Accessory tendons
may need to be transferred from the accessory digit to the
retained proper digit.
36.4.3 Ulnar Polydactyly
The surgical treatment for both types A and B ulnar polydactyly
ranges from soft-tissue excision to amputation of the polydac-
tylous digit and may require augmentation of the soft tissues of
retained digit. Functional outcomes are good, with minimal re-
habilitation considerations.
Fig. 36.7 (a) Surgical reconstruction of the collateral ligament in a 14-month-old child with Wassel type IV thumb duplication. (b) The radial duplicate
is dissected free from the preserved ulnar thumb. (c) During dissection, the radial collateral ligament of the radial thumb, along with a piece of
cartilage and periosteum, is preserved. (d) This flap of tissue is then imbricated into the radial aspect of the retained thumb. Sutures can often be
passed through the bone and periosteum surrounding the base of the metacarpal. (e) The metacarpophalangeal joint is then immobilized for 3 to 4
weeks with a K-wire placed through the joint.
Fig. 36.8 Image of a 9-year-old girl treated with the Bilhaut–Cloquet
technique for Wassel type II thumb. The reconstructed thumb shows
residual nail grooving and minimal interphalangeal joint motion as
evident by lack of skin wrinkling over joint.
Upper Extremity
336
Type A
Surgical ablation of the duplicated digit is indicated to improve
hand function and aesthetics. An elliptical incision is planned
around the digit. If a soft-tissue deficit is anticipated after abla-
tion of the digit, a racquet-type incision can be made to use
ulnar border skin of the polydactylous digit for augmenting
soft-tissue coverage of the small finger. Incisions should be
designed in a midaxial position when possible, as volar
incisions have the potential to migrate and lead to flexion con-
tractures. Exploration and division of the tendinous and neuro-
vascular structures to the extra digit is critical while preserving
the function and sensation to the remaining digit. Excision may
also require soft-tissue release, tendon balancing, joint surface
alignment, corrective osteotomies, and reattachment of intrin-
sic muscles.
Type B
Surgical excision of pedunculated digits can be performed in
the operating room using either a combination of oral sedation
with topical and local anesthetic or general anesthesia after
the age of 3 months. The polydactylous digit is placed under
tension and an elliptical incision is made at the base. The vessel
and nerve are identified. The vessel is coagulated and the nerve
is dissected and transected proximally under tension to avoid
neuroma formation. The digit is then excised completely.
Absorbable suture can be used to close the wound.
36.5 Complications
36.5.1 Radial Polydactyly
The most common complications following surgery are insta-
bility or sti ness at either the interphalangeal (IP) or
Fig. 36.9 (a–d) Example of bilateral central polydactyly with corresponding anteroposterior (AP) radiographs. Surgical treatment of central polydactyly
is complicated by differential growth of shared physis and potential for physeal injury during separation of the digits. (e) AP radiograph of both hands
at the age of 7 years. (f–h) Functional and cosmetic result. Function is complicated by minimal motion at central digit proximal interphalangeal (PIP)
and distal interphalangeal (DIP) joints, which has resulted in some scissoring of the digits.
Polydactyly
337
metacarpophalangeal (MCP) joint, grow th plate injury, and
angular deformity. First webspace narrowing, nail deformities,
and pulp deficiencies may also be seen.
Goldfarb et al reviewed the results of 31 thumbs treated for
duplication. They found significantly decreased nail width
(111% of the index nail on the operative side compared to 136%
on the nonoperative side), while girth and length were similar
between the operative and nonoperative sides. Twenty-two
thumbs demonstrated IP joint angulation, with 12 thumbs hav-
ing more than 5 degrees of angulation. IP joint angulation nega-
tively correlated with visual analog scale score.
Stutz et al evaluated 43 thumbs with an average of 17 years
after initial treatment for radial polydactyly. Eight patients
underwent reoperation an average 8 years after the initial ope-
ration. Five patients underwent IP joint arthrodesis, while three
had extrinsic tendon realignments for zigzag deformities after
type IV thumb reconstruction ( Fig. 36.10).
36.5.2 Central Polydactyly
Early physeal closure, limited digital range of motion, and joint
instability are frequent complications for type II central poly-
dactylies. Webspace and joint contractures are also common in
central polydactyly and synpolydactyly.
Wood reviewed the University of Iowa experience treating
central polydactyly over 50 years. Fifteen percent of all polydac-
tyly cases involved the ring (26) or long (8) fingers. Thirteen of
22 patients had bilateral hand involvement. A positive family
history of similar hand anomalies was present in 55% of
patients. Twenty of 22 patients had a complex syndactyly with
a type II polydactyly. Sixteen of 22 patients underwent 91 pro-
cedures. The most common complications included webspace
and flexion contractures. Fused, deviated, or nonfunctional dig-
its represented more severe complications ( Fig. 36.9).
In 1982, Tada et al published a series of 12 patients with
central polydactyly. In the series, all were type II and 10 of
12 hands had associated syndactyly. In Japan, maintaining a
five-digit hand is of paramount importance even if the digits are
nonfunctional. Therefore, in the majority of patients, the authors
removed the polydactylous digit at the first surgery and focused
on improving function during subsequent surgeries. Primary
complications included joint deviations and contractures as well
as rotatory deformities, believed to be secondary to intrinsic
muscle atrophy. The authors conclude that creation of a five-
digit hand usually led to poor functional results and they believe
a four-digit hand with removal of the hypoplastic ray should be
the goal of reconstruction in central polydactyly.
36.5.3 Ulnar Polydactyly
Most commonly, complications occur after ligation of an ulnar
polydactyly. These include (1) residual nubbin or incomplete
amputation that may or may not contain neuroma, (2) gangre-
nous digit that does not slough and requires a secondary proce-
dure for removal, and (3) infection. Watson and Hennrikus
reported infection in 1 of 21 patients, but a residual bump in
43%; yet these did not require surgical intervention. Mullick et
al reported on 10 cases of residual neuroma after ligation that
required a revision procedure for symptomatic relief.
Complications of surgical excision in type A ulnar polydactyly
include unsightly or painful scar, intrinsic tightness if transfer
was necessary, prominence of the metacarpal head, and insta-
bility of the MCP joint. However, these complications are less
common than those associated with ligation.
36.6 Conclusion
Radial polydactyly is a relatively rare condition. The di erent
types of thumb duplication are most commonly described by
the Wassel classification. The majority of cases are treated sur-
gically anywhere from 6 to 24 months of age depending on
thumb type and surgeon preference. The two main treatment
options are ablation of the hypoplastic duplicate thumb with
soft-tissue reconstruction and the Bilhaut–Cloquet procedure
(or modification). The most common complications are joint
sti ness or instability.
Central polydactyly is relatively rare. It is often associated
with syndactyly. Radiographic imaging is key to successful sur-
gical planning, which is the mainstay of management for cen-
tral polydactyly. The overall goal is to create a functional and
aesthetically pleasing hand.
Type A ulnar polydactyly is relatively uncommon, while type
B is relatively prevalent among African American patients. Type
A polydactyly is managed with surgical excision. Although con-
troversy exists over the most appropriate treatment option for
type B polydactyly, ligation is generally accepted in cases of
uncomplicated, narrow stalks.
36.7 Key Points
● Radial polydactyly is most commonly classified by the Wassel
classification, of which type IV is the most common.
● The two main surgical options for management of radial poly-
dactyly are ablation with collateral ligament reconstruction
are the Bilhaut–Cloquet procedure.
● Central polydactyly is usually associated with syndactyly and,
therefore, radiographs are key to surgical success.
● Ulnar polydactyly is classified as type A or B, with the latter
being more common, particularly among African American
patients.
● Management of type A ulnar polydactyly is usually surgical,
while type B is often managed surgically, with ligation an op-
tion in appropriate cases.
Fig. 36.10 Clinical image of a late zigzag deformity in a patient with
previous Wassel type IV thumb duplication. Long-term instability of
thumb metacarpophalangeal (MP) joint has resulted in subsequent
instability at interphalangeal and MP joints.
Upper Extremity
338
Suggested Readings
Goldfarb: A detailed technique article for treating radial polydactyly. The
manuscript addresses indications, contraindications, and complications;
2006
Stutz, et al: A review of 43 surgically reconstructed thumbs >10 years after
treatment. Two scoring systems, reoperation rates, as well as pinch strength
are reported; 2014
Mills, et al: A review of the outcomes and cost-e ectiveness of the use of a
surgical clip for type B ulnar polydactyly; 2014
Wood: A seminal paper on central polydactyly reviewing frequencies of each
type of polydactyly as well as surgical management and outcomes; 1971
Rayan, et al: A retrospective review of a large series of ulnar polydactylous
digits. Management and outcomes are discussed as well as an in-depth intro-
duction to ulnar polydactyly pathology and epidemiology; 2001
Abzug, et al: A recent review of the evidence supporting management of type
B ulnar polydactyly with a discussion on ligation of the polydactylous digit;
2013
Pritsch, et al: This article reviews and classifies type A ulnar polydactyly with
a thorough introduction to the pathology; 2013
Polydactyly
339
37 Macrodactyly
Carolyn M. Pike and Brian I. Labow
Sum m ary
Macrodactyly is a rare congenital overgrowth disorder of the
upper or lower limb. Although all tissue types are a ected vari-
ably, proportion and patterning are maintained within the
a ected territory, and structures appear grossly normal. The
disease process often follows a sensory nerve distribution
within the hand or foot. Recent genetic studies have identified
somatic mosaic gain-of-function mutations in the kinase
PIK3CA in patients with macrodactyly, as well as a number of
related overgrow th conditions. However, the basis for pheno-
typic variation, such as severity and tissue distribution of over-
growth, as well as rate of growth, remains to be established.
The diagnosis of macrodactyly should be reserved for patients
with a sporadic, isolated congenital digital enlargement a ect-
ing all tissue types not attributable to a syndrome or underlying
vascular anomaly. Current treatment options are limited to sur-
gery and can include staged debulking of soft tissue, epiphy-
siodesis, osteotomy, or ray amputation. Longitudinal growth is
far easier to correct than circumferential growth. The major
goals of treatment are to enable optimal hand function and to
provide an aesthetic limb. Minor complications of operative
treatment include hypertrophic scarring, decreased sensation,
and flexion contractures. As patients age, many will develop
progressive sti ness in the a ected digit, and some will show
radiological signs of a progressive osteoarthritis. In the future,
pharmacotherapy may provide an additional treatment option
for patients with digital overgrowth.
Keywords: macrodactyly, overgrowth, limb, congenital
37.1 Introduction
Macrodactyly is a rare congenital overgrowth disorder a ecting
the digits of the upper or lower extremity. The condition
accounts for 0.9% of all upper limb congenital anomalies. Mac-
rodactyly is sporadic, nonsyndromic, and often follows a sen-
sory nerve distribution within the hand or foot. Overgrowth is
usually noted at birth; however, both the extent and rate of
growth can vary widely. Macrodactyly can be classified by rate
of growth, with the term “progressive” describing rapid, dispro-
portionate enlargement relative to the rest of the limb, while
“static” refers to a slower, proportionate growth. Although all
tissue types are a ected variably, proportion and patterning are
maintained within the a ected territory, and structures appear
grossly normal. Another means of classification has been
through the predominant tissue type involved, for example, lip-
omatous macrodactyly for cases with gross excess of adipose tis-
sue out of proportion to other tissue types. In contrast, nerve-
territory-oriented macrodactyly has been used for digits with
enlarged nerves within the a ected zone of overgrowth that
clearly follow a sensory nerve territory. A biological distinction
between these two clinical entities has not been shown. To fur-
ther confound the situation, a wide array of descriptive terms
and eponyms has also been used to refer to macrodactyly. Given
the rarity of this condition, the lack of precision in nomencla-
ture, and our limited understanding of the pathophysiology of
this disorder, it is not surprising that there exists a paucity of lit-
erature on treatment options and long-term outcomes.
37.2 Diagnosis
In most cases, digital enlargement is obvious soon after birth.
Occasionally, minor degrees of enlargement may not be
detected until later in infancy, when continued enlargement
occurs ( Fig. 37.1, Fig. 37.2). A variety of syndromic and non-
syndromic causes for digital enlargement exist. A thorough
physical examination to look for other findings or a local or
regional cause for the overgrowth (e.g., expansile mass, vascular
anomaly) should be carefully undertaken. The diagnosis of mac-
rodactyly should be reserved for patients with a sporadic, iso-
lated congenital digital enlargement a ecting all tissue types
not attributable to a syndrome or underlying vascular anomaly.
Radiographic imaging such as ultrasonography or plain films
can be useful to document the absence of a vascular lesion and
the presence of skeletal involvement, respectively. Magnetic
resonance imaging can also be used to plan debulking proce-
dures by documenting the extent of fatty overgrowth in the
limb that frequently contributes to additional limb or digit girth
and weight. Recent genetic studies have identified somatic
mosaic gain-of-function mutations in the kinase PIK3CA in
patients with macrodactyly. These mutations have been impli-
cated in a growing number of related overgrowth conditions as
Fig. 37.1 A 13-month-old male with severe macrodactyly and syn-
dactyly of the index and middle fingers. The overgrowth includes the
thumb and adjacent palm and follows a roughly median nerve sensory
pattern.
Upper Extremity
340
well. However, the basis for phenotypic variation, such as
severity and tissue distribution of overgrowth, as well as rate of
growth, remains to be established.
37.3 Nonoperative Treatment
Since macrodactyly typically gives rise to a poorly functioning
and aesthetically displeasing digit, treatment is rarely nonoper-
ative. If the a ected digit is mildly overgrown with adequate
function and is pain free, then observation should be recom-
mended. In some cases, therapy may help with digits that are
mildly sti , though progressive arthritic changes may still
occur.
In the future, it is possible that pharmacological inhibitors of
the PI3K-AKT pathway may halt disease progression or prevent
recurrence in postoperative patients. Small clinical trials have
been initiated with drugs such as rapamycin, but there have
been limited results published. For the most part, current treat-
ment options remain limited to surgery.
37.4 Operative Treatment
Surgical treatment for patients with macrodactyly should be
individualized. The patient’s age, digit involved, type and rate of
overgrowth, as well parental and surgeon preferences will all
be factors in determining the treatment plan. The major goals
of treatment are to enable optimal hand function and to pro-
vide an aesthetic limb. In some instances, that may mean
amputation. However, a variety of procedures have been
described that reduce the length and circumferential bulk of
the digit, while maintaining sensation and circulation within
the extremity.
An overgrown digit may be salvaged via skeletal or soft-tissue
reduction, or a combination of the two. In some instances, sev-
eral procedures may be performed over time. Longitudinal
excess is far easier to correct than circumferential excess, and
it should be noted that these digits never look or function
normally ( Fig. 37.3). Adjunct procedures such as epiphyseal
growth arrest and osteotomies can also be performed to correct
angular deformities. Debulking procedures should be limited to
one side of a digit at a time to minimize the risk of vascular
compromise and usually require a staged approach.
If the digit is characterized as progressive macrodactyly with
a grossly overgrown appearance and minimal functionality that
often interferes with the other digits, amputation may be con-
sidered. Generally, a complete ray resection or transmetacarpal
amputation is required. If the thumb must be amputated to the
level of the metacarpal, a minimally a ected index ray can be
pollicized or a microvascular toe transfer can be considered.
37.5 Complications
Minor complications of operative treatment include hypertro-
phic scarring, decreased sensation, and flexion contractures.
After repeated debulking and nerve manipulation, protective
Fig. 37.2 A 15-month-old female with mild macrodactyly affecting the
index finger. The overgrowth is predominately circumferential with
minimal length difference as compared to the contralateral side.
Fig. 37.3 (a) A 7-year-old female with ulnar-sided
macrodactyly affecting the small and ring fingers
predominantly. (b) One year following soft-tissue
debulking and closing wedge osteotomy of the
small finger. The ring finger treated at another
institution was managed with partial middle and
distal phalangectomies and distal interphalangeal
joint fusion. Although the length discrepancy has
been improved, circumferential overgrowth re-
mains marked.
Macrodactyly
341
sensation is typically the result. As patients age, many will
develop progressive sti ness in the a ected digit and some will
show radiological signs of a progressive osteoarthritis. Early
arthritis in toes transferred to the hand has also been observed.
37.6 Conclusion
The rarity of macrodactyly and the highly variable phenotypic
presentation have hindered both descriptive and comparative
studies on treatment options and long-term outcomes. In gen-
eral, treatment for macrodactyly should be viewed as palliative,
although surgical therapy can certainly benefit many patients.
Depending on clinical circumstances and patient or parent pref-
erence, debulking techniques and amputation both have a role
in managing the a ected digits. Although reconstruction will
not result in a fully normal digit and often require multiple
operations, they are well-tolerated procedures and may o er a
restoration of function and aesthetics. In the near future, phar-
macological options may exist to abrogate digital overgrow th or
prevent additional growth after surgical debulking.
37.7 Key Points
● Macrodactyly is rare and characterized by congenital distal
overgrowth of the upper or lower limb. All tissue types are
a ected to a variable degree.
● Recent studies have identified somatic mosaic mutations in
the PIK3CA gene in patients with macrodactyly.
● Current treatment options are limited to surgery and can
include staged debulking of soft tissues, epiphysiodesis,
osteotomy, or ray amputation.
● In general, treatment options are well tolerated with few
minor complications; however, patients and families should
be counseled that no treatment is entirely satisfactory.
Suggested Readings
Cerrato F, Eberlin KR, Waters P, Upton J, Taghinia A, Labow BI. Presentation
and treatment of macrodactyly in children. J Hand Surg Am. 2013; 38
(11):2112–2123
Carty MJ, Taghinia A, Upton J. Overgrowth conditions: a diagnostic and thera-
peutic conundrum. Hand Clin. 2009; 25(2):229–245
Upper Extremity
342
38 Brachial Plexus Palsies
Raymond Tse and Angelo B. Lipira
Sum m ary
Pediatric brachial plexus palsies can present at birth (1 in 1000
newborns) and from blunt trauma in older children. The extent
of involvement and severity of injury is variable. This chapter
will discuss assessment, nonsurgical treatment, and surgical
treatment of brachial plexus palsy.
The approach to surgical exploration will be detailed and a
spectrum of scenarios will be presented so that the principles
of primary nerve reconstruction (including nerve graft and
nerve transfers) can be illustrated. These include upper plexus,
pan- plexus, multiple root avulsions, isolated deficits, delayed
presentation and failed reconstruction. Technical details of
nerve grafting and nerve transfers will be described.
Secondary musculoskeletal consequences of brachial plexus
palsy will also be discussed including strategies for prevention
and options for secondary surgical reconstruction.
Keywords: brachial plexus palsy, erb palsy, glenohumeral dys-
plasia, nerve graft, nerve transfer
38.1 Introduction
The brachial plexus is a complex network of nerves that provide
sensation and motor control of the upper extremity. In addition
to the intricacies of its form and functions, growth and develop-
ment in the setting of chronic denervation add another layer of
complexity to the management of pediatric brachial plexus
palsies.
An understanding of the anatomy and presentations is
important in the evaluation of patients. An appreciation of the
natural history and outcomes is critical when deciding upon
treatment. Preservation of musculoskeletal health in the setting
of chronic denervation is needed to optimize functional results.
Given the many dimensions of pediatric brachial plexus
palsy, children are best served through a multidisciplinary team
approach at specialized centers o ering coordinated, longitudi-
nal care. Early referral allows for better clinical assessment
and may reduce the chances of sequelae that a ect long-term
outcomes.
38.2 Presentation
Neonatal brachial plexus palsy (NBPP) occurs in 1/1,000 live
births and presents immediately after delivery. Downward trac-
tion on the brachial plexus results in injuries of the upper
plexus. With further traction, the zone of injury progresses to
lower regions of the plexus, ultimately resulting in pan-plexus
palsy in its most severe expression. Presentations can generally
be grouped into four patterns: type I involves C5 and C6 deficits
(Erb/Duchenne type) with loss of shoulder abduction, shoulder
external rotation, elbow flexion, and forearm supination. The
limb assumes the Erb posture due to intact antagonist muscles.
Type II involves C5–C7/C8 deficits (extended Erb type), resulting
in the additional loss of wrist extension. The limb assumes the
“waiter’s tip” posture. Type III involves deficits from C5 to C8/
T1, resulting in an arm that is generally flaccid or paralyzed.
Type IV involves C5 to T1 as well as the sympathetic chain,
resulting in flail arm with a Horner syndrome.
Upward traction on the brachial plexus results in injuries of
the lower plexus, resulting in loss of hand and wrist function
with intact elbow and shoulder function. This presentation is
known as Klumpke’s palsy and is extremely rare in NBPP.
When a root level is a ected, the site of injury tends to be at
the trunk level in the upper plexus and more proximal in the
lower plexus. This may be related to the presence of fascial
sleeves that extend from the bony vertebrae to the extraforami-
nal nerve roots in the upper plexus. This fascia is progressively
absent at more inferior nerve root levels. As such, lesions of the
lower roots are more likely to be preganglionic spinal root avul-
sions. One exception to this pattern occurs in breech deliveries,
in which C5 and C6 lesions tend to be avulsions.
Traumatic brachial plexus palsy (TBPP) can occur in older
children, but is rare. The mechanism is usually blunt injury and
most often results from motorized vehicle accidents. Compared
to NBPP, the forces involved are rapid and high energy. Conse-
quently, the patterns of injury in TBPP are varied and more
likely involve spinal root avulsions.
Injuries associated with NBPP can include fractures of
clavicle, humerus, and rarely cervical spine. Phrenic nerve
injury can occur with severe palsies, and hypoxic brain injury
can result from asphyxia. Children with TBPP should undergo a
general trauma survey.
Several conditions may present with altered limb posture or
movement and must be di erentiated from brachial plexus
palsy. “Pseudoparalysis” can result from pain related to bony
fracture; however, once healed, normal limb function resumes.
A septic shoulder may present with loss of shoulder movement,
but fever, constitutional symptoms, and a “hot joint” will often
be present. Congenital limb di erences such as arthrogryposis
can be distinguished by skeletal hypoplasia, absent skin creases
across joints, and associated anomalies. Lesions of the central
nervous system may present with altered tone, lower extremity
findings, global cognitive impairment, and/or progressive
weakness.
38.3 Assessment
38.3.1 Initial Assessment
Initial history focuses on birth, associated injuries, limb presen-
tation, and progression of recovery. Specific limb postures
should be elicited, given many families will describe a flaccid
arm in spite of intact motor functions. For example, when hand
function is present, it can be helpful to understand if the hand
function has always been present (i.e., a Type 1 palsy) or if that
function has recovered (i.e., a Type 3 palsy with persistent
upper plexus deficits). Any history of recovery helps under-
stand the type of nerve injury that has occurred. Neuropraxia
(Sunderland 1) involves no Wallerian degeneration, and func-
tion recovers rapidly. Axonotmesis (Sunderland 2) involves
Brachial Plexus Palsies
343
Wallerian degeneration and requires axon regrow th. In general,
recovery of full active range of motion within 1 month of age is
associated with no measurable long-term deficits in strength,
sensation, or limb growth. Slower recovery (i.e., Sunderland 3
and 4 injuries) is associated with greater long-term deficits.
Complete nerve disruption (Sunderland 5) results in no
recovery.
Initial examination includes an assessment of global cortical
function including age-appropriate developmental milestones,
trunk control, tone, and posturing. Neurologic examination of
lower extremities helps rule out central nervous system lesions.
Respiratory function should be assessed to screen for associated
phrenic nerve injury. The face should be assessed for Horner
syndrome (ptosis, myosis, anhydrosis) that results from disrup-
tion of the sympathetic chain. Given that the most cephalic
contribution to the sympathetic chain exits the spinal cord
along the T1 foramen, persistent Horner syndrome associated
with pan plexus palsy suggests a proximal T1 injury and possi-
bly an avulsion. Scapular stability and rhomboid function
should be assessed to determine involvement of dorsal scapular
nerve. Given the very proximal divergence of this nerve from
the brachial plexus, a deficit suggests a proximal zone of injury
that may involve a preganglionic root avulsion. Infants
with NBPP should also be assessed for torticollis, which is fre-
quently present and may be related to traction injury to neck
musculature.
Focused examination of the brachial plexus follows the
more general initial exam. Examination of infants with NBPP is
limited to observed and encouraged movements and these can
be rated according to the Active Movement Scale (AMS;