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Introduction
  • Injury to the brachial plexus during birth
    • usually a stretching injury from a difficult vaginal delivery
    • some rare reported in C-sections
  • Subtypes includes
    • Erb's Palsy (upper trunk C5-6)
      • Most common type 
    • Klumpke's Palsy (lower trunk C8-T1)
    • Total plexus injury
  • Epidemiology
    • incidence
      • approximately 1 to 4 per 1,000 live births
      • decreasing in frequency due to improved obstetric care
  • Pathophysiology
    • mechanism 
      • condition associated with
        • large for gestational age
        • multiparous pregnancy
        • difficult presentation
        • shoulder dystocia
        • forceps delivery
        • breech position
        • prolonged labor
  • Associated 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)
  • 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 M1 by 2 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 
        • C5-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
    • Toronto Scale muscle strength grading system
      • 0 - no motion
      • 1- motion present but limited
      • 2- normal motion
Erb's Palsy (C5,6) - Upper Lesion
  • Mechanism
    • results from excessive abduction of head away from 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 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
  • Physical exam
    • flaccid arm
    • both motor and sensory deficits
  • Prognosis
    • worst prognosis
Treatment 
  • Nonoperative
    • observation & daily passive exercises by parents 
      • indications
        • first line of treatment for most obstetric brachial plexopathies
      • technique
        • key to treatment is maintaining passive motion while waiting for nerve function to returr
    • Elbow Flexion Contracture
      • Serial nighttime extension splinting
        • for contracture <40 degrees
        • prevents progression, does not correct contracture
      • Serial extension casting
        • for contracture >40 degrees
  • Operative
    • early surgical attempt at nerve restoration
      • microsurgical nerve repair or nerve grafting 
        • indications
          • complete flail arm at 1 month of age
          • Horner's syndrome at 1 month of age
          • lack of antigravity biceps function between 3-6 months of age
      • neurotization (nerve transfer)   
        • indications
          • root avulsion at 3 months of age
        • donor nerves
          • sural
          • intercostal
          • spinal accessory
          • phrenic
          • cervical plexus
          • contralateral C7
          • hypoglossal
    • posterior glenohumeral dislocation - late surgery
      • open reduction and capsulorrhaphy
        • indications
          • early recognition with minimal glenoid deformity
      • proximal humeral derotation osteotomy 
        • indications
          • late recognition, no glenoid present
    • Internal rotation contractures and glenohumeral joint dysplasia - late surgery
      • latissimus dorsi and teres major transfer to rotator cuff
        • indications 
          • persistent external rotation and abduction weakness, internal rotation contractures, and mild-to-moderate glenohumeral joint dysplasia
      • pectoralis major and +/- subscapularis lengthening
        • indications
          • <5 years of age
      • proximal humeral derotation osteotomy
        • indications
          • > 5 years of age
    • forearm supination contractures - late surgery
      • biceps tendon transfers
        • indications
          • supination contractures with intact forearm passive pronation
      • forearm osteotomy (radius +/- ulna) +/- biceps tendon transfer
        • indications
          • supination contractures with limited forearm passive pronation
    • elbow flexion contractures - late surgery.    
      • Consider Anterior capsular release, biceps/brachialis tendon lengthening for severe, persistent contracture
      • May have High recurrence rate
 
 

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Questions (5)

(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%

(1874/3308)

2

Figure B

3%

(107/3308)

3

Figure C

10%

(324/3308)

4

Figure D

5%

(156/3308)

5

Figure E

25%

(816/3308)

Select Answer to see Preferred Response

PREFERRED RESPONSE 1

Figure A shows glenoid hypoplasia and retroversion which is most commonly associated with unresolved brachial plexus birth palsy.

With or without nerve repair or transfer, internal rotation contracture of the shoulder is the most common problem requiring treatment in children with incomplete brachial plexus palsy recovery. This contracture results from an imbalance between the strength of the relatively unaffected internal rotators and the paralytic external rotators. Untreated, it usually leads to progressive glenohumeral deformity characterized by posterior displacement of the humeral head on an increasingly dysplastic and retroverted glenoid.

Waters et al. performed a study to determine the effects of correction of external rotation weakness and internal rotation contractures on glenohumeral development in patients with brachial plexus birth palsy. Twenty-five patients who underwent latissimus dorsi and teres major tendon transfers to the rotator cuff were evaluated clinically and radiographically before the operation and at a minimum 2 years postoperatively. Soft-tissue rebalancing procedures alone were found to have halted the progression of, but not to have markedly decreased, glenohumeral dysplasia at the time of a 2-5 year follow-up.

Pearl et al. completed a review article on shoulder problems related to children with brachial plexus palsy. They discuss the clinical workup, imaging studies, and surgical interventions used to treat these difficult clinical problems. They also state that evaluating the status of glenohumeral development is critical throughout the treatment of these palsies.

Figure A shows an axillary cut from a CT scan of the left shoulder. There is significant glenoid hypoplasia and retroversion secondary to unresolved brachial plexus palsy.

Incorrect Answers:
Answer 2: Figure B shows absent clavicles in the setting of cleidocranial dysplasia.
Answer 3: Figure C shows a lesion consistent with an underlying diagnosis of multiple hereditary exostosis.
Answer 4: Figure D shows a widened proximal humeral physis in the setting of little leaguer shoulder.
Answer 5: Figure E shows a clinical photo of scapular winging secondary to a long thoracic nerve palsy.


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Question COMMENTS (8)

(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%

(114/2315)

2

Fixed adduction and internal rotation at shoulder with elbow extension

8%

(177/2315)

3

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

5%

(122/2315)

4

Weakness in elbow flexion

3%

(73/2315)

5

Normal physical exam without deficits

78%

(1805/2315)

Select Answer to see Preferred Response

PREFERRED RESPONSE 5

At 18 months, a majority of children who have sustained an obstetric brachial plexus birth (OBPBP) injury will have a complete recovery without weakness or noticeable asymmetry.

Neonatal obstetric birth palsy results from traction forces being applied to the arm during a difficult delivery. Brachial plexus injury is classified by the level of nerve involvement and the nature of the injury. Type I injury, or Erb’s palsy, involves C4–6 nerve roots. Type II injury, or Erb-DuChenne-Klumpke, involves the entire brachial plexus. Type III, or Klumpke palsy, involves only C8–TI. In newborns, Type 1 injuries involving the upper cord are the most common (>80%). Fortunately, most newborns with OBPBP, and almost all children that have regained elbow flexion by 3 months, will have complete recovery by 18 months of age without intervention.

Lagerkvist et al. discuss obstetric brachial plexus birth palsy (OBPBP). During a two-year period, 114 of 38,749 infants were diagnosed with a OBPBP. Only 18 children had symptoms at 18 months. Normal or near-normal muscle strength in elbow flexion, shoulder external rotation, and forearm supination at 3 months of age was almost always associated with complete recovery.

Incorrect Answers:
Answers 1-4: A majority of all brachial plexus birth palsies will have full recovery by 18 months of age.


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Question COMMENTS (2)

(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%

(447/2133)

2

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

73%

(1552/2133)

3

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

1%

(21/2133)

4

Loss of shoulder and wrist function

1%

(17/2133)

5

Ptosis, myosis and anhydrosis

4%

(85/2133)

Select Answer to see Preferred Response

PREFERRED RESPONSE 2

The findings provided are all associated with neonatal brachial plexus palsy. The best prognosis is seen in patients with the classic 'Erb palsy' presentation consisting of absent shoulder abduction and external rotation. Bicep activity is associated with a more favorable prognosis.

Kirjavainen et al evaluated the hand function of patients receiving surgery for brachial birth palsies and found that almost all patients had observable deficits in both strength and sensation at final follow up. Avulsion injuries were associated with the poorest outcomes.

Waters provides an excellent overview of the management of brachial plexus palsies. He describes the classification of palsies and associated prognosis, with poorer outcomes seen in those with more distal and advanced findings.

Wrong Answers:
Answer 1: Additional involvement of the wrist is associated with a worse prognosis, as is the rare 'Klumpke palsy' presenting with absent hand function seen in lower plexus lesions.
Answer 3: A flail extremity can be seen with preganglionic lesions as well and portends poor outcomes.
Answer 4: The additional involvement of the wrist motors is associated with a worse prognosis compared to those with shoulder involvement only
Answer 5: Preganglionic lesions are associated with the worse prognosis and are suggested when Horners syndrome or loss of rhomboid function is seen.


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Question COMMENTS (3)

(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%

(186/1610)

2

75%

7%

(110/1610)

3

50%

6%

(92/1610)

4

25%

11%

(185/1610)

5

<10%

64%

(1032/1610)

Select Answer to see Preferred Response

PREFERRED RESPONSE 5

Infants with total brachial plexus palsy (C5, C6, C7, C8, T1) with an associated Horner's syndrome have a very little (<10%) chance of ever recovering spontaneous motor function.

A study by Al-Qattan et al found that 0 of 22 infants with Horner's and total plexus palsy recovered spontaneously.

Another comparative review by Waters found Horner's syndrome was associated with very poor chances for spontaneous recovery of biceps function. Brachial plexus birth palsies are classified into upper (C5, C6, C7), lower (C8, T1), total (C5, C6, C7, C8, T1) and intermediate (predominantly C7) palsies. The presence of concurrent Horner's syndrome (ptosis, miosis, and anhidrosis on the ipsilateral side of the face) indicates injury to T1 root and the origin of the sympathetic branch that supplies the face.


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Question COMMENTS (2)

(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%

(40/878)

2

Triceps

1%

(5/878)

3

Biceps

93%

(819/878)

4

Interossei

1%

(7/878)

5

Flexor digitorum profundus

0%

(4/878)

Select Answer to see Preferred Response

PREFERRED RESPONSE 3

The biceps and brachialis muscles are supplied by C5-6 and as such a brachial plexopathy affecting the C5 nerve root would affect the child's ability to perform elbow flexion and forearm supination. The deltoid is also supplied by C5 and palsy would lead to deficient shoulder abduction. Finally, extensor carpi radialis longus and brevis are supplied by C5-6 and palsy would result in weak wrist extension.

The trapezius is supplied by the spinal accessory nerve (cranial nerve XI) and palsy would lead to scapular winging. The interossei are supplied by T1, the triceps by C6-7, and the flexor digitorum profundus by C7-8.

Waters studied the natural history of brachial plexus palsy and the effects of treatment including microsurgical repair, tendon transfer, and derotational osteotomy.

Smith et al evaluated 170 patients to determine prognosticators for recovery of function in brachial plexopathy. They found that prolonged neurological recovery or a greater level of initial injury were each associated with worse long-term shoulder function.


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