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:

  • Clavicular fracture.[26][27] Presents with visible swelling, and deformity in the clavicular region, as well as a positive "piano key sign".
  • Osteomyelitis of the humerus or clavicle.[28] Presents with high WB cell count, and osteolysis on plain radiograph.[29]
  • Septic arthritis of the shoulder.[30][31][32] Presents with soft tissue edema of the shoulder in an MRI.[31]


Epidemiology and Demographics

  • The prevalence of Erb's palsy is approximately 90 to 206 per 100,000 in the United States,[33] while prevalence worlwide is approximately 50 to 500 per 100,000 individuals.[34]
  • An incidence of 36-45 per 100,000 births has been reported for Erb's palsy in the US.[35][36]
  • There is a greater prevalence of right arm Erb's palsy over left side, with a rate of 3:1 to 7:1.[37]
  • Permanent impairment occurs in a rate of 3-25% of patients with Erb's palsy.[38]
  • In 2007, a study made by Weizsaeker et al found that african descendents had an independently higher risk for Erb's palsy.[39][40]
  • Mothers age greater than 34 years old, were found to be more prone to have a child with Erb´s palsy.[41]
  • Females seem to be slightly more affected by Erb's palsy in brachial plexus birth palsies than males,[42] 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[43]
  • Maternal obesity
  • Shoulder dystocia in prior deliveries

2. Fetal risks:

3. Labor risks:

  • Prolonged labor (second stage of labor > 60 minutes)[48]
  • Difficult extractions (need of forceps or other operative measures)[49]


Studies have shown that these typical risk factors are not relieble predictors for Erb's palsy.[50] 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.[51][52]

Interestingly, higher rate of clavicule fractures have not shown an association with BPBP,[53] but a notable association was found between BPBP and fracture of the middle third of the clavicule.[54]


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, and palmar grasp reflexes.
  • 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,[55] 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.[56]

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[57]

Common long term complications of Erb's palsy include:

  • Reduced strength and stamina[58]
  • Irregular joints function[59]
  • Muscular atrophy[60]
  • Abnormal bone growth[61]
  • Osteoarthritis[62]
  • Limb length discrepancy[63]
  • Impaired balance, and coordination[64]  

Diagnosis

Diagnostic Study of Choice

The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].

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The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].

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History and Symptoms

The majority of patients with [disease name] are asymptomatic.

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The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].

Physical Examination

Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].

Biceps reflex absent, Moro reflex with hand movement but no shoulder abduction, palmar grasp present https://www.sciencedirect.com/topics/medicine-and-dentistry/erbs-palsy

The patient is unable to abduct or externally rotate the shoulder. The patient of the supinator muscle. Sensory involvement is usually confined along the deltoid muscle and the distribution of the musculocutaneous nerve.[65] https://www.sciencedirect.com/topics/medicine-and-dentistry/erbs-palsy

waiter tip position Arm held limply adducted, internally rotated, and pronated with wrist flexed and fingers flexed (“waiter's tip” position)[66]

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Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].

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The presence of [finding(s)] on physical examination is diagnostic of [disease name].

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The presence of [finding(s)] on physical examination is highly suggestive of [disease name].


Electrophysiology/Electro diagnostics (Edx)

In the authors′ opinion, this is perhaps the single most important investigation for a plexus injury and far more valuable to make surgical and therapeutic decisions than any imaging technique. This of course pre supposes the existence of an excellent electro diagnostic department in your center which unfortunately is not always the case. The following things can be determined by Edx:

  • Type of lesion, i.e., pre or post ganglionic
  • Localization of lesion to roots trunks cords and nerves
  • Extent of the lesion
  • Status of individual muscles—denervated, reinnervating etc
  • Sequential Edx can point to recovery and help post op monitoring of results
  • Compound motor action potential (CMAP) of important nerves like the ulnar and median which are potential donor nerves in upper plexus injuries.[67]


X-ray

  • Plain X-rays for fractures and raised diaphragm (phrenic nerve injury)
  • CT myelography to determine the root status—not really done now but was the gold standard a few years ago
  • MR neurography—currently the most valuable tool for visualizing the plexus.

There are several papers discussing these modalities.[20–30] At one time CT myelography was the gold standard to decide on root avulsion in cases of adult palsy. Currently MRI is considered very useful, at least in the adults. Although there are reports of the use of MR scans the author does not routinely perform MR scans every time he sees a patient, although patients often already have one when seen by a hand and plastic surgeon. Clinical exam and electro physiology can give very adequate evidence of the status of the plexus and the indication for surgery.[68]

There are no x-ray findings associated with [disease name].

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An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

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There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

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There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

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OR

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There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

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There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Other Imaging Findings

There are no other imaging findings associated with [disease name].

OR

[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

There are no other diagnostic studies associated with [disease name].

OR

[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].

  • X-rays of the chest - to rule out clavicular or humeral fracture
  • MRI of the shoulder- may demonstrate shoulder dislocation; presence of pseudomeningoceles indicates avulsion injury of the affected spinal roots[8][9][10]
  • CT Scan of the shoulder- may demonstrate shoulder dislocation; presence of pseudomeningoceles indicates avulsion injury of the affected spinal roots[8][9][10]
  • EMG/Nerve conduction studies- presence of fibrillation potentials indicate denervation
  • https://www.physio-pedia.com/Erb%27s_Palsy


Treatment

Medical Therapy

1-Hydrotherapy: It is a form of physical therapy used because of the anti-gravity environment. It minimizes the stress on the musculoskeletal frame, allowing the neonate to move with less pain and at the same time strengthening muscles and reducing spasms. Paralyzed muscles relax in the opposite position of the waiter's tip posture by abduction at the shoulder, external rotation of the arm, and supination of the forearm. In addition, hydrotherapy helps encourage normal movements in the affected arm.[69]

2-Physiotherapy: Physiotherapy is either done alone or in combination with hydrotherapy. Response to the therapy varies from patient to patient with some healing earlier than others. Physical therapy can be required for severe cases to accompany surgery or in case of mild condition to work them through strengthening the area and healing on their own. Various forms of physical therapy exercises may include gentle stretching exercises, sensory stimulation, range of motion exercises and strength exercises.[70]

3-Occupational therapy: Occupational therapy is usually required after the surgery or for those who sustained long-term damage to help them deal with everyday activities such as eating, tying shoes, playing, drawing, and more.[71]

4-Surgery: Surgical intervention is the last resort and usually put on hold unless there is no functional recovery by physical therapy. Surgical intervention includes nerve graft and nerve decompression. Nere graft has the best chances of success.[72]

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Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Surgery

Surgery is not the first-line treatment option for patients with Erb's palsy. Surgery is usually reserved for patients depending on time and cervical roots involved and patient’s age.[73]

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.[74]

Surgical intervention is indicated if the motor function does not improve after 3 months of age. Surgical intervention is indicated if the motor function does not improve after 3 months of age.[75] https://www.sciencedirect.com/topics/medicine-and-dentistry/erbs-palsy


Surgery especially for obstetric plexus injuries was pioneered by Kennedy 1903,[31] Sever 1916[76] and Wyeth and Sharpe in 1917.[33] Kennedy in fact reported very encouraging results. However Sever's results and paper describing 1000 cases were a damper for future work for almost 50 years. Herbert Seddon revived interest in the field after world war II.[34] It was only later that Gilbert,[35] Narakas,[36] Kawabata[37] and Millesi[38] and others started the surgical treatment for both children and adults again in the 80's with remarkably impressive results.[77]


These are done in late cases when nothing else is feasible

  • Tendon transfers using available muscles
  • Trapezius transfer to stabilise shoulder
  • Shoulder and wrist arthrodesis to improve posture
  • Free Functional muscle transfer—can always be attempted as the donor muscle is uninjured and has never been denervated.[78]


Any brachial plexus injury which has not shown substantial spontaneous recovery in 3 months deserves to be explored. Timing is crucial due to the eventual loss of neuro muscular end plates at 20 to 24 months after denervation.[79]

Primary Prevention

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

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

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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/

References

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  2. Sarikcioglu L, Arican RY (2007). "Wilhelm Heinrich Erb (1840-1921) and his contributions to neuroscience". J Neurol Neurosurg Psychiatry. 78 (7): 732. doi:10.1136/jnnp.2007.115956. PMC 2117688. PMID 17575018.
  3. McGillicuddy JE (2011). "Neonatal brachial plexus palsy - historical perspective". J Pediatr Rehabil Med. 4 (2): 99–101. doi:10.3233/PRM-2011-0161. PMID 21955966.
  4. Narakas AO (1985). "The treatment of brachial plexus injuries". Int Orthop. 9 (1): 29–36. doi:10.1007/BF00267034. PMID 4018968.
  5. Menorca RM, Fussell TS, Elfar JC (2013). "Nerve physiology: mechanisms of injury and recovery". Hand Clin. 29 (3): 317–30. doi:10.1016/j.hcl.2013.04.002. PMC 4408553. PMID 23895713.
  6. Menorca RM, Fussell TS, Elfar JC (2013). "Nerve physiology: mechanisms of injury and recovery". Hand Clin. 29 (3): 317–30. doi:10.1016/j.hcl.2013.04.002. PMC 4408553. PMID 23895713.
  7. Menorca RM, Fussell TS, Elfar JC (2013). "Nerve physiology: mechanisms of injury and recovery". Hand Clin. 29 (3): 317–30. doi:10.1016/j.hcl.2013.04.002. PMC 4408553. PMID 23895713.
  8. Al-Qattan MM, El-Sayed AA, Al-Zahrani AY, Al-Mutairi SA, Al-Harbi MS, Al-Mutairi AM; et al. (2009). "Narakas classification of obstetric brachial plexus palsy revisited". J Hand Surg Eur Vol. 34 (6): 788–91. doi:10.1177/1753193409348185. PMID 19786407.
  9. Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID https://doi.org/10.1016/B0-323-03386-5/X5001-2 Check |pmid= value (help).
  10. Duff SV, DeMatteo C (2015). "Clinical assessment of the infant and child following perinatal brachial plexus injury". J Hand Ther. 28 (2): 126–33, quiz 134. doi:10.1016/j.jht.2015.01.001. PMC 4425986. PMID 25840493.
  11. Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID https://doi.org/10.1016/B0-323-03386-5/X5001-2 Check |pmid= value (help).
  12. Duff SV, DeMatteo C (2015). "Clinical assessment of the infant and child following perinatal brachial plexus injury". J Hand Ther. 28 (2): 126–33, quiz 134. doi:10.1016/j.jht.2015.01.001. PMC 4425986. PMID 25840493.
  13. Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID https://doi.org/10.1016/B0-323-03386-5/X5001-2 Check |pmid= value (help).
  14. Duff SV, DeMatteo C (2015). "Clinical assessment of the infant and child following perinatal brachial plexus injury". J Hand Ther. 28 (2): 126–33, quiz 134. doi:10.1016/j.jht.2015.01.001. PMC 4425986. PMID 25840493.
  15. Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID https://doi.org/10.1016/B0-323-03386-5/X5001-2 Check |pmid= value (help).
  16. Duff SV, DeMatteo C (2015). "Clinical assessment of the infant and child following perinatal brachial plexus injury". J Hand Ther. 28 (2): 126–33, quiz 134. doi:10.1016/j.jht.2015.01.001. PMC 4425986. PMID 25840493.
  17. Ivalde FC, Marazita-Valverde J, Bataglia D (2019). "Considerations For Surgical Planning Of Humeral Osteotomy In Brachial Plexus Birth Palsy Based On The Elbow Crease And Humeral Retroversion Measurement". J Ayub Med Coll Abbottabad. 28 (4): 479–480. PMID 31933294.
  18. Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID https://doi.org/10.1016/C2009-0-55229-4 Check |pmid= value (help).
  19. Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID https://doi.org/10.1016/C2010-0-68825-0 Check |pmid= value (help).
  20. "Erb's Palsy". Physiopedia. Retrieved 05/13/20. Check date values in: |access-date= (help)
  21. "Erb's Palsy". Physiopedia. Retrieved 05/13/20. Check date values in: |access-date= (help)
  22. "Erb's Palsy". Physiopedia. Retrieved 05/13/20. Check date values in: |access-date= (help)
  23. Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID https://doi.org/10.1016/C2010-0-68825-0 Check |pmid= value (help).
  24. Schmoldt A, Benthe HF, Haberland G (1975). "Digitoxin metabolism by rat liver microsomes". Biochem Pharmacol. 24 (17): 1639–41. PMID https://doi.org/10.1016/C2010-0-68825-0 Check |pmid= value (help).
  25. Thatte MR, Babhulkar S, Hiremath A (2013). "Brachial plexus injury in adults: Diagnosis and surgical treatment strategies". Ann Indian Acad Neurol. 16 (1): 26–33. doi:10.4103/0972-2327.107686. PMC 3644778. PMID 23661959.
  26. "Erb's Palsy". Physiopedia. Retrieved 05/13/2020. Check date values in: |access-date= (help)
  27. Peleg D, Hasnin J, Shalev E (1997). "Fractured clavicle and Erb's palsy unrelated to birth trauma". Am J Obstet Gynecol. 177 (5): 1038–40. doi:10.1016/s0002-9378(97)70010-3. PMID 9396889.
  28. "Erb's Palsy". Physiopedia. Retrieved 05/13/2020. Check date values in: |access-date= (help)
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==References== {{Reflist|2}}Template:Certain conditions originating in the perinatal period


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