Updated: 5/5/2019

Obstetric Brachial Plexopathy (Erb's, Klumpke's Palsy)

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Introduction
  • Injury to the brachial plexus during birth
    • usually a stretching injury from a difficult vaginal delivery
    • some rare cases reported following C-sections
  • Epidemiology
    • incidence
      • approximately 1 to 4 per 1,000 live births
      • decreasing in frequency due to improved obstetric care
    • often right sided or bilateral
    • risk factors
      • large for gestational age (macrosomia)
      • multiparous pregnancy
      • difficult presentation
      • shoulder dystocia
      • forceps delivery
      • breech position
      • prolonged labor
  • Associated orthopedic conditions
    • glenohumeral dysplasia 
      • increased glenoid retroversion, humeral head flattening, posterior humeral head subluxation
        • develops in 70% of infants with obstetric brachial plexopathy
        • caused by Internal rotation contracture (loss of external rotation)
    • elbow flexion contracture 
      • etiology is unclear, likely due to persistent relative triceps weakness (C7) compared with biceps (C5-6)
    • clavicle and humerus fractures
    • torticollis
  • Prognosis 
    • 90% of cases will resolve without intervention 
      • spontaneous recovery may occur for up to 2 years
    • prognostic variables for spontaneous recovery
      • favorable 
        • Erb's Palsy
        • complete recovery possible if biceps and deltoid are anti-gravity by 3 months
        • early twitch biceps activity suggests a favorable outcome   
      • poor 
        • lack of biceps function by 3 months
        • preganglionic injuries (worst prognosis)
          • avulsions from the cord, which will not spontaneously recover motor function
            • loss of rhomboid function (dorsal scapular nerve)
            • elevated hemidiaphragm (phrenic nerve)
        • Horner's syndrome (ptosis, miosis, anhydrosis)
          • less than 10% recover spontaneous motor function 
        • C7 involvement
        • Klumpke's Palsy
 
Anatomy
  • Brachial plexus diagram 
Classification
 
 Narakas Classification
Group  Roots 
Characteristics
Group I (Duchenne-Erb's Palsy) C5-C6 Paralysis of deltoid and biceps. Intact wrist and digital flexion/extension.
Group II (Intermediate Paralysis)
 C5-C7
Paralysis of deltoid, biceps, and wrist and digital extension. Intact wrist and digital flexion. 
Group III (Total Brachial Plexus Palsy)
 C5-T1 Flail extremity without Horner's syndrome
Group IV (Total Brachial Plexus Palsy with Horner's syndrome)
 C5-T1 Flail extremity with Horner's syndrome
 
Presentation General
  • Symptoms
    • lack of active hand and arm motion  
  • Physical exam
    • upper extremity exam
      • arm hangs limp at side in an adducted and internally rotated position
      • decreased shoulder external rotation 
      • affected shoulder subluxates posteriorly
    • provocative testing
      • stimulate neonatal reflexes including Moro, asymmetric tonic neck and Votja reflexes
      • pain with gentle shaking of a flail arm may indicate pseudoparalysis from infection or fracture rather than nerve palsy
    • Hospital for Sick Children Active Movement Scale (AMS) muscle strength grading system 
      • full range of motion with gravity eliminated (score of 4) must be achieved before higher scores may be assigned
Imaging
  • Radiographs
    • may be useful for evaluation of clavicle or humerus fractures
    • limited utility in infant given minimal ossification of humeral head and glenoid
    • axillary view to evaluate position of humeral head if patient is older and suspicion is high for joint subluxation
  • Myelography/CT myelography/MRI
    • may be used to distinguish between root avulsion and extraforaminal rupture
  • EMG/NCV
    • poor reliability and often underestimate the severity of injury
  • Ultrasound
    • allows for assessment of joint subluxation or dislocation
Erb's Palsy (C5,6) - Upper Lesion
  • Most common type  
  • Mechanism
    • results from lateral flexion of the head towards the contralateral shoulder with depression of the ipsilateral shoulder producing traction on plexus
      • occurs during difficult delivery in infants
  • Physical exam 
    • adducted, internally rotated shoulder; pronated forearm, extended elbow (“waiter’s tip”)
    • C5 deficiency
      • axilllary nerve deficiency
        • deltoid, teres minor weakness
      • suprascapular nerve deficiency
        • supraspinatus, infraspinatus weakness 
      • musculocutaneous nerve deficiency
        • biceps and brachialis weakness 
    • C6 deficiency
      • radial nerve deficiency
        • brachioradialis, supinator weakness 
  • Prognosis
    • best prognosis for spontaneous recovery
Klumpke's Palsy (C8,T1) - Lower lesion
  • Mechanism
    • rare in obstetric palsy
    • usually arm presentation with subsequent traction/abduction from trunk 
  • Physical exam
    • deficit of all of the small muscles of the hand (ulnar and median nerves)
    • “claw hand”
      • wrist in extreme extension because of the unopposed wrist extensors
      • hyperextension of MCP due to loss of hand intrinsics
      • flexion of IP joints due to loss of hand intrinsics
  • Prognosis
    • poor prognosis for spontaneous recovery
    • frequently associated with a preganglionic injury and Horner's Syndrome
Total Plexus Palsy (C5-T1) 
  • Mechanism
    • stretch, rupture, and avulsion injury
  • Physical exam
    • flaccid arm
    • both motor and sensory deficits
  • Imaging
    • chest radiograph to look for ipsilateral hemidiaphragm paralysis from phrenic nerve injury 
  • Prognosis
    • worst prognosis
Treatment  - General
  • Nonoperative
    • observation & daily passive exercises by parents 
      • indications
        • first line of treatment for all obstetric brachial plexopathies while awaiting return of function
      • key to treatment is maintaining passive motion while waiting for nerve function to return
  • Operative
    • microsurgical nerve grafting  
      • indications
        • lack of antigravity biceps function between 3-9 months of age
        • postganglionic injury with intact nerve roots with segmental injury to nerve
      • outcomes
        • improved outcomes are seen with shorter grafts (<10cm)
    • nerve transfer or neurotization
      • definition
        • nerve transfer refers to fascicles from one nerve transferred into a nother nerve that supplies a muscle
        • neurotization refers to placing nerve fascicles directly into a neuromuscular junction of a muscle
      • indications
        • lack of antigravity biceps function between 3-9 months of age
        • preganglionic injury or avulsion of nerve roots
Treatment - Shoulder Dislocation & Contractures
  • Operative
    • soft tissue procedures
      • latissimus dorsi and teres major transfer  (Hoffer procedure)
        • indication
          • persistent internal rotation contracture or external rotation weakness without glenohumeral dysplasia
        • technique
          • pass tendons posteriorly around humerus to create external rotation forces 
      • pectoralis major and +/- subscapularis lengthening
        • indication
          • to lessen the internal rotation forces
        • may be used in conjunction with tendon transfers
      • arthroscopic release for internal rotation contractures
    • bony procedures
      • proximal humeral derotation osteotomy (Wickstrom) 
        • indication
          • persistent internal rotation contracture or external rotation weakness with glenohumeral dysplasia 
      • arthrodesis
        • indication
          • non-functional deltoid with good function of hand and wrist
Treatment  - Elbow Flexion Contracture
  • Nonoperative
    • serial nighttime elbow extension splinting 
      • indications
        • for elbow flexion contracture <40 degrees
      • outcomes
        • prevents progression, does not correct contracture
    • serial elbow extension casting
      • indications
        • for elbow flexion contracture >40 degrees
  • Operative
    •  anterior capsular release, biceps/brachialis tendon lengthening
      • indications
        • for severe, persistent contracture
      • outcomes
        • may have high recurrence rate
Treatment  - Forearm
  • Operative
    • indications
      • residual supination contracture of the forearm
    • technique
      • biceps rerouting tendon transfer
        • intact passive passive pronation
      • forearm osteotomy with biceps rerouting tendon transfer
        • limited passive forearm pronation
Treatment  - Wrist and hand
  • Operative
    • indications
      • replace function for a paralyzed muscle
    • force is preportional to cross-sectional area of the muscle
    • amplitude is proportional to the length of the muscle
    • technique
      • tendon transfers
        • wrist drop
          • pronator teres to ECRB
        • loss of finger extension
          • FCR or FCU to EDC 2-5
        • thumb abduction
          • EIP to abductor pollicis brevis
Complications
  • initial nerve inury
    • phrenic nerve palsy
      • if persistent may require diaphragm plication
  • surgery
    • shoulder tendon transfers
      • radial and axillary nerve palsies
  • phrenic nerve palsy
    • if persist may require diaphragm plication
 

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Questions (16)

(OBQ13.97) An infant first presents holding his limb in the position shown in Figure A. Birth history reveals that he was a large-for-gestational age infant and labor was prolonged. His orthopedist recommends passive stretching including shoulder abduction and external rotation. For which of the following scenarios is this an appropriate line of treatment? Review Topic

QID: 4732
FIGURES:
1

One-month-old infant with partial antigravity biceps strength.

84%

(2311/2740)

2

Four-month-old infant with scapula winging and elevated hemidiaphragm.

3%

(93/2740)

3

Seven-month-old infant with posterior glenoid dysplasia.

3%

(74/2740)

4

Eight-month-old infant with recent anterior Z-lengthening of the subscapularis tendon.

5%

(128/2740)

5

Five-month-old infant with recent ipsilateral latissimus dorsi and teres major transfer.

4%

(110/2740)

ML 2

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PREFERRED RESPONSE 1

(OBQ13.12) A 3-month-old infant holds his limb in the position seen in Figure A. Examination reveals winged scapulae, and absent rhomboid, rotator cuff and latissimus dorsi function. Which is the most appropriate treatment plan? Review Topic

QID: 4647
FIGURES:
1

Instruct the parents to perform passive shoulder abduction and external rotation, and elbow flexion exercises and reassess at 6 months of life.

62%

(1298/2079)

2

Neuroma resection and sural nerve grafting

6%

(117/2079)

3

Neuroma resection and direct brachial plexus repair

6%

(135/2079)

4

Nerve transfer to biceps and brachialis branches of the musculocutaneous nerve using fascicles from median and ulnar nerves.

24%

(500/2079)

5

Latissimus dorsi transfer

1%

(20/2079)

ML 5

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PREFERRED RESPONSE 4

(SAE07PE.35) In obstetrical brachial plexus palsy, which of the following signs is associated with the poorest prognosis for recovery in a 2-month-old infant? Review Topic

QID: 6095
1

Persistent inability to bring the hand to the mouth with the elbow stabilized at the side

22%

(8/36)

2

Persistent inability to actively abduct the arm past 90 degrees

6%

(2/36)

3

Persistent inability to externally rotate the shoulder past 20 degrees

0%

(0/36)

4

Persistent unilateral ptosis, myosis, and anhydrosis

69%

(25/36)

5

History of clavicle fracture at birth

0%

(0/36)

ML 3

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(SAE07PE.10) An 18-month-old boy with obstetric brachial plexus palsy is being evaluated for limited right shoulder motion. Physical therapy for the past 6 months has failed to result in improvement of the contracture. Which of the following studies is necessary prior to any shoulder reconstruction? Review Topic

QID: 6070
1

Electromyography

59%

(377/636)

2

MRI of the shoulder joint

35%

(224/636)

3

MRI of the brain

2%

(10/636)

4

Radiograph of the elbow

3%

(16/636)

5

Aspiration of the right shoulder

1%

(6/636)

ML 5

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(OBQ12.160) Which of the following is the most common long term consequence of untreated brachial plexus birth palsy? Review Topic

QID: 4520
FIGURES:
1

Figure A

57%

(2027/3560)

2

Figure B

3%

(115/3560)

3

Figure C

9%

(330/3560)

4

Figure D

5%

(167/3560)

5

Figure E

25%

(885/3560)

ML 4

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PREFERRED RESPONSE 1

(OBQ12.218) A newborn child born via a difficult breech delivery is found to have a brachial plexus birth palsy. While initially born with a flail limb, the child regained elbow flexion at 10 weeks. At age 18 months (1.5 years old), which of the following deficits is most likely to be seen on physical exam? Review Topic

QID: 4578
1

Claw hand

5%

(125/2577)

2

Fixed adduction and internal rotation at shoulder with elbow extension

8%

(217/2577)

3

Hyperextension of the MCP joints and flexion of the IP joints of the hand

5%

(130/2577)

4

Weakness in elbow flexion

3%

(78/2577)

5

Normal physical exam without deficits

78%

(2001/2577)

ML 3

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(OBQ10.73) An infant is born with total brachial plexus palsy and Horner’s syndrome after a difficult vaginal delivery. What is the prognosis for spontaneous recovery of motor function in the involved arm by 3 months?
Review Topic

QID: 3161
1

>90%

12%

(206/1765)

2

75%

7%

(116/1765)

3

50%

5%

(95/1765)

4

25%

11%

(199/1765)

5

<10%

65%

(1142/1765)

ML 3

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(OBQ08.232) Which of the following muscles would be affected if a 6-month-old child sustains a birth-related brachial plexopathy affecting C5 nerve root? Review Topic

QID: 618
1

Trapezius

5%

(54/1103)

2

Triceps

1%

(9/1103)

3

Biceps

93%

(1022/1103)

4

Interossei

1%

(8/1103)

5

Flexor digitorum profundus

0%

(5/1103)

ML 1

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(OBQ11.232) A 3-month-old is brought to clinic for evaluation of a right upper extremity abnormaility present since birth. Which of the following physical exam findings is associated with the best functional outcome? Review Topic

QID: 3655
1

Loss of hand function with preserved shoulder function

21%

(484/2348)

2

Absent shoulder abduction and external rotation with intact wrist and digit flexion and extension

73%

(1712/2348)

3

Rotator cuff dysfunction, elevated hemidiaphragm, and absence of rhomboid function

1%

(26/2348)

4

Loss of shoulder and wrist function

1%

(26/2348)

5

Ptosis, myosis and anhydrosis

4%

(89/2348)

ML 2

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PREFERRED RESPONSE 2
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