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Erb-Duchenne Palsy
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ANATOMY
The brachial plexus lies between the neck and the axilla with the distal
portion lying behind the clavicle and the pectoral muscles. It is formed from the C5,
C6, C7, C8 and T1 nerve roots and is best understood by dividing it into three parts;
trunks, divisions and cords. The upper trunk is formed from the C5 and C6 roots,
the C7 root becomes the middle trunk and the lower trunk is formed by the C8 and
T1 roots. Each trunk then divides into an anterior and a posterior division giving
six divisions that unite to form cords at the level of the clavicle. The three posterior
divisions unite to form the posterior cord, the anterior divisions of the upper and
middle trunks form the lateral cord while the anterior division of the lower trunk
carries on to form the medial cord. The major peripheral nerves of the upper limb
are formed from the cords in the following way:
● The posterior cord gives rise to the radial and axillary nerves;
● The lateral cord gives rise to the musculocutaneus nerve; and
● The medial cord forms the ulnar nerve. The median nerve is formed from
branches of the medial and lateral cord. The other branches of the cords are shown
in table 1 and figure 1.
There are three branches that arise proximal to the cords and these are the dorsal
scapular, the suprascapular and the long thoracic nerve (figure 1).
Functional Anatomy
The most classically used statistical correspondences of the myotomes is
Bonnel and Rabischong’s. However, there is great anatomical variability. Several
nerve roots participate in the formation of a nerve. A function can depend on several
roots and a rootcan participate in several functions.
Briefly, C5C6 injury manifests by a deficit in abduction and lateral rotation
of the shoulder and by a deficit in elbow flexion and supination. If the injury extends
to the C7 root, a deficit in elbow, wrist, and finger extension and a deficit in radial
inclination are associated. Injury to the C8T1 roots results in a deficit in flexion of
the wrist and fingers as well as the intrinsic function of the hand.
Clinical Anatomy
Correlating the physical findings on clinical examination of the upper limb
with a clear understanding of the anatomy of the brachial plexus allows the clinician
to determine the level of the lesion. The proximal shoulder girdle muscles receive
innervation from the level of the roots, trunks and cords, while innervation from the
arm onwards comes from the terminal branches. The shoulder muscles are
predominantly supplied by C5 and C6 spinal roots, while C7 contributes to the
elbow, wrist and finger extensors; C8 and Tl contribute to the long finger flexors
and intrinsic muscles of the hand. Table I highlights some of the major upper limb
muscles along with their nerve supplies.
In addition to identifying the level of nerve root injury, it is as important to
try todetermine the location of the nerve lesion. The lesion may occur at any point
from the origin at the spinal cord to the terminal branches, and may be associated
with the death of some ventral horn neurons following injury. Neonates are more
susceptible to motor neuron death than adults, and proximal lesions are more likely
to produce motor neuron death than distal ones. Proximal lesions are termed
preganglionic lesions, also known as root avulsions. These lesions occur proximal
to the dorsal root ganglia (DRG) (Fig. 2). Surgical repair of these lesions is
technically not possible due to the absence of a proximal stump to attach to, and
because performing laminectomies on infants in order to gain better exposure of the
roots is not recommended. More distal lesions are termed postganglionic lesions or
nerve ruptures. Postganglionic lesions may be complete ruptures, neuroma in
continuity, simple neurapraxia, or a combination of the above. Neuroma in
continuity is the most cornmon finding during brachial plexus reconstruction (Fig.
2).
Several clinical findings suggest a root avulsion. For example, abnormal
head tilting to the uninjured side due to unopposed pull of the contralateral
paraspinous muscles may be a soft sign of upper root avulsion. Winging of the
scapula indicates a proximal injury involving the long thoracic nerve originating
from C5, C6, and C7 near the root origins from the foramina, and may be associated
with root avulsions. Phrenic nerve injury (C4 and C5) is also associated with
proximal root avulsion. The cervical sympathetic chain is closely related to the C8-
T 1 nerve roots and the stellate ganglion receives white rami communicantes from
C8 and T 1(7). With C8- TI root avulsions, the input from C8 and Tl to the stellate
ganglion is disrupted, resulting in Homer's Syndrome (myosis, ptosis and
anhydrosis).
EPIDEMIOLOGY
Despite progress in obstetrics, the incidence of brachial plexus birth palsy
has remained stable over the last few decades. This may be essentially related to the
unpredictability of shoulder dystocia and the increase in mean birth weight.The
incidence of brachial plexus birth palsy varies greatly between series and is
estimated between 0.04 and 0.4% of live births. The largest epidemiological survey,
conducted at the national level in the United States in 2008, reported a 0.15%
incidence of live births. The frequency of the anterior left occipitoiliac presentation,
which places the right shoulder under the maternal pubis, explains the
predominance of these lesions on the right side. In 4% of cases,the lesions can be
bilateral. Proximal C5C6 paralysis (Erb-Duchenne), by far the most frequent,
accounts for 50–60% of cases. In approximately 30% of cases,proximal C5C6
involvement is extended to the C7 root. Total C5T1 paralysis, a severe condition
often with consequential sequelae, is found in 15–20% of cases. Distal C8T1
paralysis (Klumpke-Déjerine), occurring during breech delivery, is exceptional and
accounts for less than 2% of cases.
CLINICAL SYMPTOMS
The characteristic deformity of the C5-C6 injury of brachial plexus includes
winged scapula, adduction and medial rotation at the shoulder. When C7 is involved
the elbow is in the extension and the forearm is in pronation. There is flexion and
ulnar deviation of the wrist and the fingers are flexed. The deep tendon reflexes of
the affected muscles (biceps, triceps, and brachioradialis) may be absent or
diminished.
PATHOGENESIS
Injury Mechanism
The most common mechanism is stretching of the brachial plexus during
the second stage of a dystocic delivery, either through traction of thehead (cephalic
presentation) or traction on the upper limb (breech presentation). The causes are
probably multifactorial, but the two main risk factors are dystocia of the shoulders
and macrosomia. Delivery with instrumentation (forceps/ vacuum extractor),
prolonged labor, primiparity, prematuriry (breech deliveries); a history of brachial
plexus birth palsy during a preceeding birth, an excessive maternal weight gain are
also a potential risk factors.
In very rare cases, the brachial plexus can be streched during the initial
phase of delivery during the passage of the fetus at the sacral promontory. Finally,
certain uterine anomalies have been reported (fibroma, bicornuate uterus) to be
potentially responsible for intrauterine involvement in the brachial plexus.
Four anatomical characteristics explain the chronology, the type, and the
seat of brachial plexus lesion:
Orientation of the brachial plexus: the C5 and C6 roots take a nearly
vertical dscending direction, which makes them more vulnerable
than the distal roots that are nearly horizontal. Excessive traction on
the brachial plexus will first lead to proximal root involvement and
then secondary involvement of the distal roots. Consequently,
proximal lesions are more frequent;
The posterosuperior ligament: it is present at the C5 and C6 roots
and anchors these two roots to the transverse apophysis,
thusprotecting them from radicular avulsions. At the C7C8T1
roots,when this ligament is absent, any traction force is transmitted
directly to the rootlets. This explains the frequency of distal avul-
sions and the infrequency of distal avulsions;
Conjunctive tissue: a protective element, conjunctive tissue is richer
at the trunks than at the roots, which explains the more frequent seat
of these lesions in the roots;
Pre-fixed brachial plexus: the participation of the C4 branch can be
considered as a predisposing factor for proximal lesions, whereas
the post-fixed plexus (participation of a T2 branch) instead plays
aprotective role for the distal roots.
Types of lesions
To determine the severity of the problem, it is important to distinguish two
types of the lesions (Fig.2):
The pre-ganglion or avulsion lesion, located upstream of the dorsal
root ganglion, is a veritable tearing of the rootlet at the spinal cord.
This lesion, which most often involves the C8 and T1 roots, is
particularly serious because it cannot be repaired by direct surgery.
It should be systematically sought on MRI or CT-myelography for
better surgical planning;
The post-ganglion lesion is located downstream of the dorsal root
ganglion. Three types are described in the Sunderland classification:
Type 1 or Seddon neurapraxia: a simple elongation of the brachial
plexus without interrupting the nerve continuity, leading to
transitory paralysis that is spontaneously resolved,
Type 2 or axonotmesis: partial nerve rupture touching the axon but
keeping the nerve sheath intact. Spontaneous recuperationis possible
but with a risk of a “switching” error,
Type 3 or neurotmesis: complete nerve rupture with
neuromaformation. Spontaneous recuperation is impossible but the
lesion remains accessible to nerve repair.
DIAGNOSIS
The Clinical Examination
Diagnosis of brachial plexus birth palsy at birth is generally easy and based
solely on clinical examination.
Questioning covers the obstetrical history looking for risk factors. Elements
of dystocic delivery are often found. Macrosomia with a birth weight over 4 kg is
frequently associated.
Inspection of the newborn finds asymmetric active movements between the
two upper limbs with an overall spontaneous position in adduction and internal
rotation. One must immediately search for signs of severity, most particularly the
Horner syndrome triad: ptosis, myosis, and enophthalmia (Fig. 3).
Additional Investigations
Initial Phase
In the initial phase, the clinical exam is crucial both to establish the
diagnosis and to assess lesion severity. Therefore, no complementary exam is
indicated here. However, a standard radiological workup will be useful in searching
for associated lesions. Most particularly, an X-ray of the scapular girdle and the
humerus should be requested to search for any associated clavicle or humerus
fracture, which may not be visible on the X-ray if there is physeal injury. A chest
X-ray can be requested if phrenic paralysis is suspected. In this case, it shows
araised homolateral hemidiaphragm associated with inertia on the radioscopic
exam.
DIFFERENTIAL DIAGNOSIS
It is mainly the physeal injury of the proximal humerus that can cause a
problem in the differential diagnosis with brachial plexus birth palsy. This
lesion,responsible for painful unilateral pseudoparalysis, is not visible on the X-ray
because the epiphysis has not yet ossified (Fig. 8).
Microsurgical Indications
Microsurgical intervention aims to improve function, often without the
expectation that the affected extremity will completely recover. General consensus
is that microsurgical reconstruction should be undertaken for infants with global
lesions and Horner’s syndrome, by 3 months of age. As noted previously, without
microsurgical intervention, these patients have lifelong profound functional
deficits. Early timing of surgery is important in global lesions to minimize motor
endplate loss and maximize time for recovery. There has been some argument that
the criteria for early intervention should be expanded to include patients with initial
fail extremity with some recovery of biceps, but no significant hand or forearm
recovery.24 Conversely, patients with upper plexus injuries who achieve recovery
of biceps by 3 months are treated without microsurgical intervention.
The most controversial element of brachial plexus management remains the
timing of surgery for patients with rupture-type injuries, in which there are varying
degrees of severity of injury and recovery. Gilbert and Tassin, as well as other
surgeons, adopted the absence of return of biceps function by 3 months as an
indication for microsurgical intervention. They cited poorer shoulder outcomes at
5 years and increased likelihood for secondary procedures in patients who regained
biceps function after 3 months. Other authors have adopted more conservative
management, citing that absent elbow flexion alone at 3 months can incorrectly
predict a poor recovery and may lead to unnecessary microsurgical intervention.
These authors report that patients who regained biceps function between 4 and 6
months of age were able to achieve global shoulder function with secondary tendon
transfers comparable to the function of those who underwent microsurgical
procedures at 3 months of age. In 1 study, the 5- to 6-month biceps recovery
transition seemed to best demarcate outcome, but ultimately, the exact timing is still
unknown.
PROGNOSIS
In the literature, the natural history of brachial plexus birth palsy is still
debated. Selection bias, the heterogeneity of the brachial plexus lesions, and the
diversity of the evaluation measurements explain the great variability of these data.
Most authors agree that brachial plexus lesions are most often
transitory,with 75–95% of cases advancing to complete recuperation. The most
recent studies report a lower rate of 66%, with a residual deficit in 20–30% and
considerable alteration of function in 10–15% of cases.
The final prognosis of brachial plexus birth palsy is directly related to the
type of initial nerve lesions. Although the extent of the lesions can be assessed
clinically, today no examination can specify the type of lesion (simple elongation
rupture, or avulsion). The prognostic assessment at birth is based only on clinical
criteria. Total paralysis and the presence of Horner syndrome are the main factors
announcing a poor prognosis. The prognostic value of diaphragm paralysis is,
however, controversial. It is often cited in publications as a factor of poor prognosis.
In a study analyzing the prognostic value of phrenic nerve involvement, Al-Qattan
et al. observed that it had only a low predictive value, with 13% poor results. In
our practice, the cases of brachial plexus birth palsy associated with phrenic
paralysis –including two complicated cases of respiratory distress requiring
hospitalization, were all proximal and all progressed favorably on the neurological
level.
After birth, it is the quality and most particularly the speed of recuperation
that will provide the greatest amount of information on prognosis and will
consequently guide the therapeutic indications. This requires that the clinical exam
be repeated within the outpatient clinic at least once a month. The assessment of
recuperation is based for the most part on biceps muscle testing, which remains,
particularly for the first 6 months, the simplest and the most reliable monitoring
indicator. The main objective is to identify the cases in which early nerve repair
could contribute to a better functional result than conservative treatment.