Algorithm for treatment of children of first months of life with brachial plexus birth palsy

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Abstract


Aim. We present the algorithm for treatment designed specially for medical doctors who are involved in treatment process of children with brachial plexus birth palsy during first few months of their life.

Materials and methods. We analyzed domestic and foreign literature which highlights the problem of brachial plexus birth palsy.

Results. Key-moments of diagnostic and treatment of these patients were discovered. Based-upon them algorithm was developed.

Conclusion. Integration of developed algorithm in clinical practice is essential for understanding the etiology, pathogenesis, natural history, differential diagnostic and prior treatment by medical doctors of different specialties to improve the quantity of medical service.


Brachial plexus birth injury varies in severity (from mild sprains to spinal root avulsion) as do the clinical symptoms (from mild to plegia). This disorder occurs at a frequency of 0.38–5.1 per 1,000 live births, predominantly in full-term infants. The upper trunk of the brachial plexus (C5–C6 roots) is injured in most cases and is in combination with the middle trunk (C7) injury is found in a 30% of patients. Isolated injury of the lower trunk (C8–T1 roots) is very rarely observed (1% of cases). The total type of brachial plexus injury (C5–T1) is observed on average in 15% of cases. Patients with this condition completely recover during the initial months of life in 70%–80% of cases, and functionally significant palsy persists in 20%–25% of cases. The need for neurosurgical intervention is considered in the absence of recovery by the age of 3–4 months. The absence of a unified algorithm for early support in such children may contribute to development of major joint contractures and significant limitations of upper limb function [1-8].

The main objectives of medical care for children with brachial plexus birth injury during the initial months of life include prevention of joint contractures in the upper limb and careful monitoring for a timely decision on the need for neurosurgical treatment.

Etiology

  1. Fetal shoulder dystocia (big fruit/contracted pelvis).
  2. Abnormal fetal postition during labor (occipital, pelvic, tilting of the hand).
  3. Tractional and rotational obstetric procedures (midwifery maneuvers, forceps, and vacuum extraction) [9-14].
  4. Neuropraxia (nerve compression, stretch, ischemia, local damage to the myelin sheath, undamaged axons, and transient conduction block): fully reversible condition, which does not require a surgical intervention.
  5. Axonotmesis (damage to the axon and inner nerve sheath): possible spontaneous recovery.
  6. Neurotmesis (violation of the integrity of the entire cross section of the nerve): spontaneous recovery is rarely complete, and in some cases, neurosurgical intervention is indicated.
  7. Avulsion (root separation from the spinal cord): spontaneous recovery is impossible, and if no neurosurgical intervention is performed, the prognosis for recovery of upper limb function is poor [15].

Pathological types of brachial plexus damage

Clinical manifestations of brachial plexus injury in the initial months of life are presented in Table 1 [2, 3, 16, 17].

Table 1. Clinical manifestations of brachial plexus injury

Sign

Upper type 
(Erb–Duchenne)

Lower type 
(Dejerine–Klumpke)

Complete type (Kehrer)

Injury level

Upper (± middle) trunk of brachial plexus or C5–C6 roots (± C7)

Lower trunk of brachial plexus or C8–Th1 roots

C5–Th1

Flaccid paralysis/upper extremity palsy

Shoulder girdle and proximal parts: brachial adductors and rotators (deltoid, supra-/infraspinatus muscles); flexors of the forearm (biceps) and supinators. C7 — extensors of the forearm (triceps), wrist, and fingers

Distal parts: palmar interosseous and lumbrical muscles of the wrist; flexor muscles of the fingers; and, more rarely, the flexors of the wrist

Proximal and distal parts

Extremity position

Adduction and internal rotation of the shoulder, arm is extended (if the damage is at C7, there is moderate flexion), forearm is in pronation, and the hand is flexed and turned posteriorly

Passive position of the wrist and fingers, wrist is flattened, and fingers are in a bird-like position

Hand is dropped along the body; there is no specific pathological pattern

Active movements

Limited or absent: abduction and external rotation of the shoulder and supination and flexion of the forearm; C7— extension of the forearm, wrist, and fingers

Preserved in the fingers and wrist

Limited or absent in the wrist and fingers

Preserved in the shoulder and elbow joints

Do not exist in all joints of the upper extremity

Muscle tone

Reduced in the proximal parts

Reduced in the distal parts, and wrist is flattened

Reduced in all parts

Passive movements

Free, painless

Deep reflexes

Reduced with biceps and triceps

Carporadial reflex is reduced or absent

Cannot be induced

 

Reflexes in newborns

Babkin reflex1 is absent; Moro’s2 and grasping reflexes (±)

Reduced or absent Babkin, Moro’s, and grasping reflexes (±)

Babkin, Moro’s, and grasping reflexes are absent

Sensitivity

Can be reduced on the outer surface of the shoulder and forearm

Can be reduced on the inner surface of distal parts

Reduced

Ventral support

Drop hand with paresis

Possible accompanying symptoms

Dystonic torticollis (head turns to the affected shoulder), palpation tenderness at Erb’s point3, Kofferate syndrome4

Trophic disorders of the upper extremity (edema, cyanosis, cold skin, etc.) and Horner’s syndrome5 on the affected side

Horner’s syndrome on the affected side and trophic disorders

1Babkin reflex: pressure on the region of the tenor → mouth is opening and the head is turning in the direction of the irritated hand

2Moro’s reflex: suddenly let the child’s hands move outward when they are opening → extension and abduction of the arms followed by flexion and adduction

3Erb’s point: located posterior to the sternocleidomastoid muscle above the clavicle

4Kofferate syndrome: paresis of the diaphragm due to S4-root or phrenic nerve injury

5Horner’s syndrome: ptosis (drooping of the eyelid), myosis (pupil constriction), and enophthalmos (retraction of the eyeball)

Indirect signs of root avulsion

  1. Horner’s syndrome.
  2. Symptoms of spinal cord lesions (recovery of reflexes, increased tone in the lower extremity on the affected side).

Differential diagnosis

  1. Clavicle fracture: swelling of the soft tissues, limited amplitude of active movements in shoulder and elbow joints, swelling, deformity in the clavicle region, anxiety on palpation, passive movements in the shoulder joint, wrist and forearm functionality are normal, pathological position of the upper extremity is absent, and 3–4 days after birth there is a palpable callus.
  2. Injury of the spine and spinal cord due to birth trauma: vertebral symptoms (torticollis, hyperextension of the head, back tension of the neck muscles and palpation tenderness, and tenderness and limitation of cervical spine movements) + symptoms of spinal cord injury.
  3. Fracture of the humerus: absence of active movements and sharp cries when testing passive movements in shoulder and elbow joints, deformation, and swelling in the area of the fracture. Epiphysiolysis of the proximal/distal epiphysis of the humerus: absence of active movements and tenderness of passive movements in the shoulder/elbow joint and movement in the wrist and fingers are not restricted/restricted and swelling and blueness of soft tissue in the shoulder/elbow joint.
  4. Osteomyelitis and arthritis: delayed occurrence of symptoms and limitation of movement range in the joints of the upper extremity (pseudoparesis and antalgic posture), pain when performing active and passive movements, local symptoms (swelling, tenderness, redness, and hyperthermia of soft tissue), fever, leukocytosis, and left deviation in differential white cell count.
  5. Parrot’s disease in congenital syphilis: limited amplitude of active movements in joints of the upper extremity, pain in passive movements, history of syphilis in the mother/she is not examined, and positive serum tests (e.g., RW) in the mother and newborn. Pain and limited range of movements associated with the occurrence of multiple microfractures, either confirmed or suspected, require a gentle clinical examination and careful attitude to the child.
  6. Sprengel’s disease (malformation of the shoulder girdle and high standing of the scapula)—asymmetry of scapula positions (elevation on the affected side), scapula deformation, and limited amplitude of passive abduction of the upper extremity. Active shoulder movements are preserved but limited (primarily retraction and rotation are not affected).
  7. Nontraumatic disorders of the spine and spinal cord (tumors, malformations, etc.).
  8. Congenital malformations of the upper extremity.

Treatment and diagnostic strategy in the early neonatal period (in the maternity hospital)

  1. History of childbirth (large fetus, difficulty passing the shoulders, abnormal fetal position during delivery, and obstetric traction procedure)
  2. Careful supervision by a neonatologist (in the case of cervical spine birth injury, there is a risk of respiratory, visceral, and vegetative disorder impairment)
  3. Examination by a neurologist (if available in maternity hospitals)
  4. Examination by an orthopedic surgeon (if available in maternity hospitals)
  5. Transcranial and transfontanellar ultrasonography (to exclude intracranial traumatic injury)
  6. Skull ultrasonography if indicated (external signs of head injury, including hematoma of the scalp, crepitus, and depression).
  7. Ultrasonography of the cervical spine and spinal cord (without clinical signs of a cervical spine birth injury)
  8. Psychosocial support to the mother
  9. Gentle care

Treatment and diagnostic strategy in the early neonatal period (in the maternity hospital)

  1. History of childbirth (large fetus, difficulty passing the shoulders, abnormal fetal position during delivery, and obstetric traction procedure)
  2. Careful supervision by a neonatologist (in the case of cervical spine birth injury, there is a risk of respiratory, visceral, and vegetative disorder impairment)
  3. Examination by a neurologist (if available in maternity hospitals)
  4. Examination by an orthopedic surgeon (if available in maternity hospitals)
  5. Transcranial and transfontanellar ultrasonography (to exclude intracranial traumatic injury)
  6. Skull ultrasonography if indicated (external signs of head injury, including hematoma of the scalp, crepitus, and depression).
  7. Ultrasonography of the cervical spine and spinal cord (without clinical signs of a cervical spine birth injury)
  8. Psychosocial support to the mother
  9. Gentle care
  10. Immobilization of the upper extremity during the first 5–7 days after birth (rest and reduce tension of the cervical roots) by fixing the hands to the anterior abdominal wall with support bandage (mesh tubular elastic bandage; see Fig. 1).
  11. In case of paresis combined with an upper extremity with cervical vertebral syndrome: lateral position with elevated head end and immobilization of the cervical spine—cotton and gauze dressing or cervical collar (strictly lateral position, heart rate control, risk of apnea, and aspiration!)
  12. Isolated paresis of the upper extremity: obtain a discharge report from a maternity home within the standard deadlines for supervision by a neurologist and an orthopedic surgeon at the place of residence. In the case of concomitant paresis, provide referral to specialists depending on the concomitant pathology.
  13. In case of severe paresis/plegia or Horner’s syndrome, the patient should be transferred to a children’s hospital for further examination, treatment, and consultation with a neurosurgeon.

Therapeutic and diagnostic strategy during the late neonatal period and initial months of a child’s life (in outpatient or inpatient settings)

  1. Supervision by a neurologist on a monthly basis
  2. Supervision by an orthopedic surgeon monthly for the first 4 months of life (evaluation of reactive muscle function recovery of the upper extremity and early diagnosis of emerging contractures)
  3. Consultation with a neurosurgeon in the neonatal period for suspected avulsion (plegia + Horner’s syndrome) [16, 18]
  4. Therapeutic exercises starting from the 7th–10th days of life should be a priority, with the main goal of preventing formation of contractures [19]. The treatment is focused on the healthy upper extremity, and the range of movements in the joints of both upper extremities should be the same.
    - passive movements in the joints of a paralyzed arm (parents should perform the movements 7–10 times a day, and each exercise should be repeated at least 10 times), including shoulder —flexion and abduction to 170°, external rotation with movement toward the body, and abduction to 90°; forearm—flexion/extension, pronation/supination, and extension of wrist and fingers. A set of exercises are available here:http://www.rch.org.au/uploadedFiles/Main/Content/plastic/BRACHIAL_PLEXUS_book.pdf. [20].
    - stimulation of active movements in a paralyzed arm by means of toys, materials with different textures, swimming, and objects at different temperatures in a safe range.
  5. Massage: from the age of 2 weeks—frottage; from the age of 1 month—differentiated (for a paralyzed muscle—stimulation and for an unaffected muscle—relaxation); punctate; and from 1.5 months—in combination with vibration.
  6. Physical therapy (secondary importance; efficacy has not been proven): UHF, photochromotherapy (blue matrix), magnetotherapy on the lateral surface of the neck, and supraclavicular region on the affected side; from the age of 1 month—paraffin or ozokerite applications on the extremity for 10–15 min at a temperature of 37°C–39°C, wool wrapping, and electrophoresis with neostigmine methylsulfate (neostigmine on the dorsum of the wrist, C5–Th1—nicotinic acid); and segmental EHF [21].
  7. Electromyography (EMG) and stimulation electroneuromyography have some information value in assessing the nature of the nerve injury for children <1 month old; in some cases, needle EMG is performed to determine the number of intact motor units and the stage of the denervation and re-innervation process (prescribed by a neurologist or a neurosurgeon) [22-26].
  8. In the absence of active forearm flexion by the age of 3–4 months, the decision on performing neurosurgical treatment should be made.


Irina A Kriukova

North-Western State Medical University n. a. I.I. Mechnikov; Saint Petersburg State medical affiliation “Maternity hospital № 10”

Author for correspondence.
Email: i_krukova@mail.ru

Russian Federation MD, PhD, neurologist, sonologist, assistant of the chair of pediatric traumatology and orthopedics. North-Western State Medical University

Nikita O Khusainov

The Turner Scientific and Research Institute for Childrens Orthopedics

Email: i_krukova@mail.ru

Russian Federation MD, PhD student. The Turner Scientific and Research Institute for Children’s Orthopedics.

Alexei G Baindurashvili

North-Western State Medical University n. a. I.I. Mechnikov; The Turner Scientific and Research Institute for Childrens Orthopedics

Email: turner01@mail.ru

Russian Federation MD, PhD, professor, corresponding member of RAS, honored doctor of the Russian Federation, Director of The Turner Scientific and Research Institute for Children’s Orthopedics. Head of the chair of pediatric traumatology and orthopedics of North-Western State Medical University n. a. I.I. Mechnikov

Galina A Ikoeva

North-Western State Medical University n. a. I.I. Mechnikov; The Turner Scientific and Research Institute for Childrens Orthopedics

Email: ikoeva@inbox.ru

Russian Federation MD, PhD, assistant professor of the chair of pediatric neurology and neurosurgery. North-WesternStateMedicalUniversity n. a. I.I. Mechnikov. Chief of the department of motor rehabilitation. The Turner Scientific and Research Institute for Children’s Orthopedics

Yuriy E Garkavenko

North-Western State Medical University n. a. I.I. Mechnikov; The Turner Scientific and Research Institute for Childrens Orthopedics

Email: yurijgarkavenko@mail.ru

Russian Federation MD, PhD, professor of the chair of pediatric traumatology and orthopedics. North-Western State Medical University n. a. I.I.Mechnikov, leading research associate of the department of bone pathology of The Turner Scientific and Research Institute for Children’s Orthopedics

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Copyright (c) 2016 Kriukova I.A., Khusainov N.O., Baindurashvili A.G., Ikoeva G.A., Garkavenko Y.E.

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