Erb Palsy


Article Author:
Hajira Basit
Citra Dewi Ali


Article Editor:
Neal Madhani


Editors In Chief:
Rodrigo Kuljis
Oleg Chernyshev
Aninda Acharya


Managing Editors:
Avais Raja
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Khalid Alsayouri
Frank Smeeks
Kristina Soman-Faulkner
Trevor Nezwek
Radia Jamil
Patrick Le
Sobhan Daneshfar
Anoosh Zafar Gondal
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Heba Mahdy
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Nazia Sadiq
Hajira Basit
Phillip Hynes
Tehmina Warsi


Updated:
6/4/2019 7:23:18 PM

Introduction

Erb palsy, or Erb-Duchenne paralysis, is a paralysis of the arm caused by the injury to the upper group of the main nerves supplying it, specifically the upper trunk C5-C6 of the brachial plexus.  It is one of the most common neurological birth injuries and these injuries most commonly, though not exclusively, arise from the traction on the neck during difficult childbirth. According to the severity of the injury, it can either resolve on its own over some time or may require rehabilitative therapy and surgery. [1]

Etiology

Erb palsy relates to an injury of cervical spinal nerves C5 and C6. C5-C6 nerves form part of brachial plexus which comprises of ventral rami of cervical spinal nerves C5-C8 and thoracic nerve T1. These nerves network from the spine and pass through the cervicoaxillary canal in the neck and the ribs and emerge into the axilla. Most commonly the brachial plexus is injured by traction of the neck during delivery. The most important risk factors are macrosomia and shoulder dystocia. It usually occurs in macrosomic infants who needed to be pulled from the birth canal. This maneuver causes stretching of the brachial plexus which may injure it.  However, brachial plexus injury can also result independently of shoulder dystocia and average weighing neonates.[2]. Other risk factors that lead to brachial plexus injury include breech delivery, an ultrashort second stage of labor, multipara mother, maternal obesity, maternal diabetes, or vacuum and forceps delivery. Erb palsy may also occur in cesarean section. [3][4][5][6][1]

Epidemiology

It is a common birth injury with estimated prevalence ranges of 0.9 to 2.6 per 1000 live births. It causes significant arm weakness that affects 0.4 to 5 in 10,000 births. 

Pathophysiology

The ventral rami of the cervical spinal nerves C5 and C6 join to form the upper trunk of the brachial plexus. Each trunk is divided into anterior and posterior divisions which further divided to create the cords then branches namely axillary nerve, suprascapular nerve and musculocutaneous nerve to supply the muscle of the upper limb. Injuries to the brachial plexus range from mild to severe. The degree of severity can be defined as neuropraxic, axonotometric, or neurometric. A neuropraxic injury is the least severe. It is reversible and heals without any complication. Axonometric lesions involve disruption of axon and myelin sheath. Recovery of axonometric lesions depend on the level of lesion, and it may take months to heal with proper treatment including physiotherapy. Neurometric has the worst prognosis, with avulsion of the roots from the spinal cord causes irreversible damage affecting the axon, myelin sheath as well as the supporting structures through the nerve. The proximal end of the nerve tries to regenerate by forming a neuroma.[2]

History and Physical

Erb palsy most commonly presents with a history of birth injury. The signs of Erb's palsy include loss of sensation in the arm and paralysis and atrophy of the deltoid, biceps and brachialis muscles. The muscular damage results in the classical sign of Erb’s palsy called the ‘waiter’s tip hand’. The neonate presents with the arm hanging limply from the shoulder with adduction at the shoulder, internal rotation of the upper arm, pronation of the forearm, an outward direction of the palm, giving the appearance of "waiter's tip posture.". The arm cannot be raised from the side and all power of flexion of the elbow is lost due to the paralysis of the biceps muscle. Moro reflex is absent on the affected side. Meanwhile, the grasp reflex is present which differentiates it from another brachial nerve palsy, Klumpke palsy (C7, C8, and T1). The neonate may have impaired sensation on the outer side of the arm and/or phrenic nerve palsy (C3, C4, and C5a) resulting in hemidiaphragm paralysis.[2]

Evaluation

Erb palsy is a disorder of clinical diagnosis, but some interventions can confirm it. MRI of the brachial plexus and cervical cord is likely the best imaging technique if required. An MRI can also exclude any cancer or tumor that may affect the brachial plexus. An X-ray of the baby's shoulder excludes any fractured bone or problems with the shoulder or elbow joints. Electromyography (EMG) results can estimate and record the electrical activity of the muscle with absent fibrillations likely indicate neuropraxia. Nerve conduction studies (NCS) can measure the time required for the electrical stimulus to move through a particular nerve.  CT myelography works by injecting the contrast material to give detail of neonate spinal cord and nerve root.[7][2]

Treatment / Management

The management of Erb's palsy depends upon its severity with some cases requiring surgical intervention while others can be managed by physiotherapy alone. Recommended treatment includes early immobilization followed by passive and active range of motion exercises. Different treatment modalities suggested for the Erb's Palsy are:

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.

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.

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.

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.[8][9][9]

Differential Diagnosis

Erb palsy should be differentiated from other injuries such as Klumpke injury, clavicular fracture, cervical ribs, osteomyelitis of the humerus or clavicle and septic arthritis of the shoulder.

-In the case of Klumpke injury, there is paralysis of the forearm and hand muscle due to injury in C7, C8, and T1.  The neonate presents with "claw hand" due to injury to the flexor muscles of the wrist, fingers, and forearm pronator. It also affects the intrinsic muscles. The neonate injury also may be associated with Horner syndrome due to the affection of T1 which will affect the dilators of iris and elevators of the eyelid. 

-A clavicular fracture can cause pseudoparalysis which can be mistaken for Brachial plexus injury.

-Cervical ribs may predispose to Erb’s palsy by the stretching of nerves around the cervical rib or through concentrated pressure when the shoulder is forced against the cervical spine

-Osteomyelitis of the humerus or clavicle and septic arthritis of the shoulder can occasionally lead to a BPI most likely due to thrombophlebitis of the vasa vasorum or an arterial embolism causing ischemic nerve damage. 

Prognosis

Erb's palsy generally has a favorable prognosis and can be completely resolved in the first year of life if recovery begins with four weeks. However, if the muscle remains dennervated then without reinnervation, the injury becomes irreversible at 18 to 24 months. These time-sensitive, irreversible changes are the scientific basis for early management recommendations. [10]

Complications

Neglected cases may lead to 20% to 30% permanent deficit of the nerve function. A persistent Erb’s palsy may also affect the child’s quality of life and self-esteem. Many long term complications of Erb's palsy due to impaired innervation are: 1) decreased strength and stamina, 2) abnormal movement and function of joints, 3) muscular atrophy, 4) impaired bone growth, 5) osteoarthritis, 6) limb length discrepancy, 7)impaired balance, and coordination.  

Postoperative and Rehabilitation Care

The timing of rehabilitation is controversial. The effectiveness of rehabilitation timing to the functional outcomes of the patient can be evaluated using Modified Mallet Scale (MMS). Rehabilitation is the best choice of treatment before surgical procedures in mild to a moderate degree regardless of age. 

Consultations

Several paradigms may bring disadvantage to the neonate including “all neonatal brachial plexus palsy recovers,” “wait a year to see if recovery occurs," and "do not move the  arm." These approaches by far lead to 20% to 30% deficit of total function of the nerves affected. Thus early recognition and interference are necessary beginning with proper diagnosis and including rehabilitation and, in certain cases, surgical intervention.

Deterrence and Patient Education

Erb palsy requires early diagnosis and management for the best outcomes. Prenatal counseling of diabetic mothers to have good glycemic control is important to reduce the risk of Erb's palsy due to macrosomia and shoulder dystocia. Parents of the affected children should be counseled about the importance of physiotherapy at an early age for the functional recovery of the arm as well as surgical repair in case of sustained injury.

Pearls and Other Issues

About 10% of infants with neonatal brachial plexus palsy fail to gain functional recovery. Intervention for this group is needed and includes the resection of neuroma-in-continuity (NIC) and surgical nerve reconstruction to solve the problem. Formation of NIC inhibits functional recovery of neonatal brachial plexus palsy. A series of NIC tissue samples are obtained and analyzed using an array of immunohistochemical techniques. It is discovered that NIC contained multiple focal globular areas with markedly diminished myelination. This focal area, focal myelin deficits (FMDs), includes Schwann cells that wrap axons in normal configuration but do not form the myelin sheath. Axon segments in FMDs also had disturbed nodes of Ranvier. The axon in FMDs may be a pathobiological basis for explaining conduction blocks in neonatal brachial plexus that leads to failure of functional recovery. Otherwise, this mechanism needs further studies.

Enhancing Healthcare Team Outcomes

Erb palsy requires multidisciplinary care involving the parents, primary care physicians, nerve surgeons, physiatrists, and occupational and physical therapists for the optimal rehabilitation for those with residual deficits. About 90% of infants with Erb palsy will show recovery with time, but 10% will continue to have a dysfunctional arm. The key is to prevent this injury in the first place.[2]


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Erb Palsy - Questions

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A mother presents to the hospital for a follow-up visit of her neonate born two weeks ago. The baby was breech, and his head could not be delivered at that time which led to the use of forceps resulting in Erb's palsy of the right arm. The baby has been gaining weight appropriately, but the mother is excessively worried about the prognosis of the condition. Which of the following is true about Erb palsy?



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A newborn, after having undergone a very difficult delivery, is found to have an abnormal left arm. It appears to be hanging by the side and rotated medially. The forearm is extended and pronated. The infant does not appear to move his arm and the Moro reflex is absent. What is the diagnosis?



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A 6-month-old infant was brought to the hospital for his physiotherapy session. His right arm is limping by his body. He was a macrosomic baby born to a diabetic mother with poor glycemic control. The childbirth was particularly challenging due to shoulder dystocia resulting in a birth injury. Which neurological damage is suspected if the upper extremity shows the waiter's tip position?



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After a difficult delivery a neonate is noted to have asymmetric Moro reflex. One of the arms is held in adduction, shows internal rotation at the shoulder, is pronated and extended at the elbow, and wrist flexion. Which nerve roots are most likely affected?



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A 30-year-old male hiker presents to the hospital with a recent history of fall. He was climbing a peak when he slipped. He appears anxious as he is unable to move his right arm. On physical examination, he is keeping his right arm by his body, and it is rotated inwards. Injury to the superior trunk of the brachial plexus is suspected. Which of the following would be the most essential step when assessing a patient who has Erb palsy?



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An infant born two hours ago by spontaneous vaginal delivery develops respiratory distress. The pregnancy was only remarkable as the mother was diabetic. There is cyanosis, labored, irregular breathing, and decreased breath sounds on the left side. The left arm has decreased tone. Oxygen is started and chest radiograph is normal. Select the best diagnostic study.



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A 30-year-old primigravida presents to the hospital for a 32nd-week prenatal visit. She was diagnosed with gestational diabetes at 28th week via screening. She has poor glycemic control. On her recent ultrasound, there is suspicion of macrosomia. The clinician counsels her about the possible complications of poor glycemic control during pregnancy including birth defects, difficult labor and Erb Duchenne palsy. Which of the following is the greatest risk factor for Erb palsy in this patient?



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Erb Palsy - References

References

Bourque PR,Warman Chardon J,Bryanton M,Toupin M,Burns BF,Torres C, Neurolymphomatosis of the Brachial Plexus and its Branches: Case Series and Literature Review. The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques. 2018 Mar;     [PubMed]
Bykowski J,Aulino JM,Berger KL,Cassidy RC,Choudhri AF,Kendi AT,Kirsch CFE,Luttrull MD,Sharma A,Shetty VS,Than K,Winfree CJ,Cornelius RS, ACR Appropriateness Criteria{sup}®{/sup} Plexopathy. Journal of the American College of Radiology : JACR. 2017 May;     [PubMed]
Raducha JE,Cohen B,Blood T,Katarincic J, A Review of Brachial Plexus Birth Palsy: Injury and Rehabilitation. Rhode Island medical journal (2013). 2017 Nov 1;     [PubMed]
Evans-Jones G,Kay SP,Weindling AM,Cranny G,Ward A,Bradshaw A,Hernon C, Congenital brachial palsy: incidence, causes, and outcome in the United Kingdom and Republic of Ireland. Archives of disease in childhood. Fetal and neonatal edition. 2003 May     [PubMed]
Chater M,Camfield P,Camfield C, Erb's palsy - Who is to blame and what will happen? Paediatrics & child health. 2004 Oct     [PubMed]
Acker DB,Gregory KD,Sachs BP,Friedman EA, Risk factors for Erb-Duchenne palsy. Obstetrics and gynecology. 1988 Mar     [PubMed]
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