Erb's palsy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Moises Romo, M.D.,


Overview

Duchenne-Erb's palsy, or simply Erb's palsy, is a lesion to the C5-C6 brachial roots; can be a form of brachial plexus birth palsy (BPBP), or can be aquired in adulthood. It occurs when there is a stretching of the superior brachial plexus (neuropraxia, neuroma, neurotmesis), leading in most of the cases to a temporal weakness and loss of sensation in certain muscles of the upper extremity.

Historical Perspective

Erb's palsy, was first discribed by William Smellie, a British obsterician, in 1754 on his midwifery book, "Traité de la theorie et pratique des accouchemens".[1]

In 1861, French neurologist, Guillaume Benjamin Amand Duchenne was the first to discover the association of paralysis in the same muscles (deltoid, biceps, and subescapularis) of arms and shoulders of infants delivered vaginally, naming it "obstetric palsy of the brachial plexus".

In 1874, German neurologist, Wilhelm Heinrich Erb concluded that paralysis was associated with a radicular lesion at the level of the superior plexus,C5-C6, and not from isolated peripheral nerve lesions.[2][3]

In 1985, Narakas made a classification for Erb's palsy grading the severity and determening the prognosis.[4]


Classification

Erb's palsy may be classified according to the severity of damage produced to the brachial plexus nerves as:

  1. Neurapraxia: Mild, temporal disruption or compresion of the myelin sheet, with no structural damage to the axon.[5]
  2. Axonotmesis: Anatomic interruption of the myelin sheath and the axon of the nerve, but perneurium and epineurium remain intact.[6]
  3. Neurotmesis: Complete tear of the nerve, including the axon with his endoneurium, perineurium, and epineurium.[7]


Erb's palsy, in turn, is also classified within the Narakas system, wich categorize Brachial Plexus Birth Palsy (BPBP) according to the roots envolved, and its directly linked to its prognosis:[8]

  • Group I: Classic Erb's palsy (C5-C6 roots). Good spontaneous recovery in > 80% of cases.[9][10]
  • Group II: Extended Erb's palsy (C5-C7 roots). Good spontaneous recovery in > 60% of cases.[11][12]
  • Group III: Global palsy without Horner syndrome (C5-C8 roots). Good spontaneous recovery of shoulder/elbow in > 30–50% of cases. Functional hand frequently noted.[13][14]
  • Group IV: Global palsy with Horner syndrome (C5-T1). Without surgery severe arm deficits are expected.[15][16]


Pathophysiology

Erb's palsy is caused by damage to the upper brachial plexus, cervical roots C5-C6,[17] and in 50% of the cases, involving C7.[18][19] Although, damage can occur at any time, this usually happens during a delivery complicated by shoulder dystocia.[20] Excesive upper traction to the baby's head when shoulder beign down produces stretching to the nerve fibers that can produce a simple temporal disruption or compresion, to a complete tear of the entire plexus.[21] Another way of damage to upper brachial plexus can be made by excessive pressure on the baby's raised arm during a breech delivery. [22]

Superior trunk of the brachial plexus has motor and sensory fibers. Palsy of C5 and C6 roots affects movement of deltoid, biceps, brachialis, infraspinatus, supraspinatus, and serratus anterior muscles, as well as sensation of the skin of the shoulder, anterolateral forearm, index and thumb fingers.[23] Consequently, the patient is unnable to abduct or externally rotate the shoulder, as well as supinate the forearm because of weakness.[24]


Causes

The most common cause of Erb's palsy is a difficult extraction during vaginal delivery due to shoulder dystocia. To review the risk factors that aim into a difficult extraction, and ultimately promote Erb's palsy, click here.

In adults, the most frequent causes are motor vehicule accidents (>90% of cases), work-related trauma (industry), severe fall with stretching of neck, assault (knife or bullet injury), and iatrogenic injury (surgery).[25]

Differentiating Erb's palsy from other Diseases

Erb's palsy must be differentiated from:

  • Klumpke palsy. Presents with "claw hand" and negative grasp reflex, many times accompanied by Horner's syndrome.[26]
  • Clavicular fracture.[27][28] Presents with visible swelling, and deformity in the clavicular region, as well as crepitance and a positive "piano key sign".
  • Osteomyelitis of the humerus or clavicle.[29] Presents with high WB cell count, and osteolysis on plain radiograph.[30]
  • Septic arthritis of the shoulder.[31][32][33] Presents with soft tissue edema of the shoulder in an MRI.[32]


Epidemiology and Demographics

  • The prevalence of Erb's palsy is approximately 90 to 206 per 100,000 in the United States,[34] while prevalence worlwide is approximately 50 to 500 per 100,000 individuals.[35]
  • An incidence of 36-45 per 100,000 births has been reported for Erb's palsy in the US.[36][37]
  • There is a greater prevalence of right arm Erb's palsy over left side, with a rate of 1.3:1 to 7:1,[38][39] with only 3% affecting both arms.[40]
  • Permanent impairment occurs in a rate of 3-25% of patients with Erb's palsy.[41]
  • In 2007, a study made by Weizsaeker et al found that african descendents had an independently higher risk for Erb's palsy.[42][43]
  • Mothers age greater than 34 years old, were found to be more prone to have a child with Erb´s palsy.[44]
  • Females seem to be slightly more affected by Erb's palsy in brachial plexus birth palsies than males,[45] while this proportion greatly inverts in adulthood, with more accidents occuring in males.

Risk Factors

Common risk factors for neonatal Erb's palsy involve maternal, fetal, and labor factors:

1. Mothers risks:

  • Maternal diabetes[46]
  • Maternal obesity
  • Shoulder dystocia in prior deliveries

2. Fetal risks:

3. Labor risks:

  • Prolonged labor (second stage of labor > 60 minutes)[51]
  • Difficult extractions (need of forceps or other operative measures)[52]


Studies have shown that these typical risk factors are not relieble predictors for Erb's palsy.[53] Furthormore the majority Erb's palsy newborns have comed from mothers with no risk factors, and even C-section does not completely eliminate the risk for neonatal Erb's palsy.[54][55]

Interestingly, higher rate of clavicule fractures have not shown an association with BPBP,[56] but a notable association was found between BPBP and fracture of the middle third of the clavicule.[57]


Screening

As mentioned previously, the majority Erb's palsy cases appear in children of mothers with no risk factors, for this reason, the condition is imposible to predict and becomes a challenge for the entire team to reduce this risk to the minimum. However, prenatal care can help us know when a vaginal delivery may be complicated:

  • Pelvic measurment. Will inform us if maternal pelvis is narrow and its form (gynaecoid, anthropoid, platypelloid, android), wich could complicate a delivery.
  • Serum glucose measurements. Will inform us of maternal diabetes, wich is directly associated with macrosomic children.
  • Prenatal ultrasound. Will inform us the fetal presentation and the approximate fetal weight.

Once child is delivered, there are several things that can make us suspect an Erb's palsy:

  • Negative ipsilateral Moro reflex, and positive palmar grasp reflex.
  • Muscle tone and movement scale.


Natural History, Complications, and Prognosis

Prognosis is generally good; If left untreated, <25% of neonates Erb's palsy may progress to develop permanent disability and impairment, while the rest will recover spontaneously after a month,[58] depending on the severity of the injury (close to 100% of neuropraxias).

Denervated muscles become irreversibly injured after 18 to 24 months, and these time sensitive changes become the basis for early management.[59]

Several scales have been developed to predict the prognosis in Erb's palsy: Toronto test score, active movement scale, mallet scale, and Toddler Arm Use Test[60]

Common long term complications of Erb's palsy include:

  • Reduced strength and stamina[61]
  • Irregular joints function[62]
  • Muscular atrophy[63]
  • Abnormal bone growth[64]
  • Osteoarthritis[65]
  • Limb length discrepancy[66]
  • Impaired balance, and coordination[67]  

Diagnosis

Diagnostic Study of Choice

The diagnosis of Erb's palsy is made clinically, but there are several studies that may help to confirm it or rule out other conditions, such as MRI, electromyography (EMG), x-rays, nerve conduction studies (NCS), and CT myelography.[68]

History

The classic history of a newborn with Erb's palsy is a prolonged vaginal delivery of a macrosomic child with shoulder dystocia, product of a diabetic mother.

Physical Examination

Patients with Erb's palsy usually appear on physical examination with the affected arm held limply adducted, internally rotated, and pronated with an outward direction of the palm, wrist flexed, and clinged fingers (“waiter's tip” position).[69] The patient is unable to abduct or externally rotate the shoulder. Sensory involvement is usually confined along the deltoid muscle and the distribution of the musculocutaneous nerve.[70] Biceps, brachioradialis, and Moro reflexes are absent with hand movement and palmar grasp present (diferentiating factor from Klumpke palsy).[71] Sensation is impaired in the skin of the shoulder, anterolateral forearm, index and thumb fingers.[23] Ocasionaly, there can be phrenic nerve palsy leading to hemidiaphragmatic paralysis.[72][73]

In prolonged Erb's palsies it could be observed an atrophy of the deltoid, biceps, and brachialis muscles.[74]

Electrophysiology/Electro diagnostics (Edx)

Electrophysiology studies are probably the single most important imaging study for Erb's palsy, and an important tool when making surgical and therapeutic decisions.[75] Among the aplications for these kinf of studies are that they help cathegorize the localization, extent, and type (preganglionic/ postganglionic) of lesion, postoperative monitoring, the status of individual muscles (denervated, reinnervating etc), and can give you a compound motor action potential (CMAP) diagnosis.[75]

X-ray

Help to rule out shoulder dislocations, clavicular, humeral, and rib fractures, as well as accesory cervical ribs, and raised diaphragm (phrenic nerve injury).[76][75]

CT scan

CT may detect the same findings as X-rays with greater precision, as well as pseudomeningoceles, wich could be a sign of nerve root avulsions.[77][78]

CT myelography used to be the gold standard for evaluation of nerve roots avulsion in adults, but now is rarely used.[75]

MRI

MRI can detect same findings as CT scan, with greater sensitivity for soft tissues.

EMG/Nerve conduction studies

EMG can detect the presence of fibrillation potentials indicate denervation.[78]


Treatment

Medical Therapy

There is no consensus on the appropiate treatment for Erb's palsy, since this will depend on the severity of the afection, age of the patient, and prognosis.[79] Treatment is often multidisciplinary, and aimed to be as conservative as possible.[80]

  • Immobilization and splinting. Is commonly used during the first week from injury to avoid flexion contractures.[81]
  • Electrostimulation. Its use is still controversial.[82]
  • Hydrotherapy. It is a usefull method of physical therapy, due to its anti-gravity properties, facilitates muscle relaxation, and range of motion; at the same time, increases strength, and muscle build-up due to water resistance.[83]
  • Physiotherapy: It can be done in combination with hydrotherapy or alone. Consist in pasive and active range of motion, strengthening, and streching exercises.[84]
  • Occupational therapy. Is usually requiered for adult patients with long-term damage to help them deal with daily-living activities (eating, tying shoes, playing, drawing).[85]

Surgery

Surgery is not the first-line treatment option for patients with Erb's palsy. Surgery is usually reserved for patients with no functional recovery from physical therapy, and will depend on the time from damage, the cervical roots involved, and the patient’s age.[86]

In infants, surgical intervention is indicated if the motor function does not improve after 3 months of age.[87] After 20 to 24 months of denervation, exists a loss of neuro-muscular end plates, wich makes timing for surgery a crucial point.[88]

Surgical intervention includes nerve decompression, nerve repairs, and graft implantation, with great chances of success.[89]

One of the surgical procedures done for persistent cases is Hoffer-procedure, wich has been shown to improve functional outcomes when operated before age 2.5 years.[90]


Secondary reconstruction procedures have been realized in patients as a last resource to gain partial functionality,[91] these include:

  • Pectoralis major release to improve passive range of motion[92]
  • Tendon transfers for decreased active external rotation[93]
  • Trapezius transfer to stabilise shoulder[94]
  • Fractional release of the pronator teres and the lacertus fibrosis to improve passive supination[95]
  • Shoulder and wrist arthrodesis to improve posture[96]


Primary Prevention

There are no established measures for the primary prevention of [disease name].

OR

There are no available vaccines against [disease name].

OR

Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

OR

[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].


Placing the mother in the ‘McRoberts’ position. This is when she is placed on her back and her legs are removed from stirrups. Two people are required to flex each of the mother’s leg backwards at the same time towards the mother’s head to widen the pelvis.

3. If Step 2 is not effective, while still in the McRobert’s position, a third person should apply suprapubic pressure by pressing down just above the maternal pubic bone in an effort to encourage the fetal shoulder to descend down into the pelvis and under the bone. Gentle traction should be applied to deliver the baby.

https://www.roydswithyking.com/solicitors-for-life/medical-negligence-claims/birth-injury-claims/erbs-palsy-claims/erbs-palsy-info/causes-of-erbs-palsy/

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==References== {{Reflist|2}}Template:Certain conditions originating in the perinatal period


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