Index of Suspicion in the Nursery

An Enigma of Recurrent Extubation Failure in

1 a Neonate

Ayesha Romana, MD,* Indhuja Rajarathinam, MD,* Prathik Bandiya, DM,*

Rajendra Shinde, MD,* Niranjan Shivanna, MD,* Naveen Benakappa, MD*
*Indira Gandhi Institute of Child Health, Bangalore, India

PRESENTATION

A 10-day-old male neonate is referred in view of inability to wean off respiratory

support. The infant is born through assisted breech delivery with a birthweight of
2.75 kg to a gravida 2 woman. Antenatal history is not significant. However, there
is meconium staining of the amniotic fluid. The infant did not cry after birth
and his Apgar scores are 3 and 6 at 1 minute and 5 minutes, respectively. He
has cyanosis and tachypnea soon after birth requiring intubation. Vital signs
at admission include a temperature of 97.7°F (36.5°C), heart rate of 155 beats/
min, capillary refill time of 2 seconds, and saturation of 94% on ventilator.
The infant’s activity is diminished, with poor tone in all 4 limbs.
The possibilities considered at this stage are perinatal asphyxia, meconium
aspiration syndrome, congenital pneumonia, congenital heart disease, sepsis, and
persistent pulmonary hypertension of the newborn.
Initial investigations indicate negative sepsis screen (total white blood cell
count 20,000/mL [20109/L], C-reactive protein <6 mg/L [57.1 nmol/L], abso-
lute neutrophil count 15,800/mL [15.8109/L]); normal blood glucose (98
mg/dL [5.4 mmol/L]); hemoglobin 10.7 g/dL (107 g/L); ionized calcium 4.8 mg/dL
[1.2 mmol/L]; and normal blood gas analysis (pH 7.37, PCO2 39 mm Hg [5.2 kPa], PO2
73 mm Hg [9.7 kPa], bicarbonate 22 mEq/L [22 mmol/L]). Chest radiography shows
NOTE The editors and staff of NeoReviews normal lung fields bilaterally with fracture of right clavicle (Fig 1).
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AUTHOR DISCLOSURE Drs Romana,

Rajarathinam, Bandiya, Shinde, Shivanna, and
Benakappa have disclosed no financial
relationships relevant to this article. This
commentary does not contain a discussion
of an unapproved/investigative use of a
commercial product/device. Figure 1. Radiograph showing normal position of diaphragm (on ventilator).

Vol. 20 No. 11 N O V E M B E R 2 0 1 9 e663

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 CASE PROGRESSION

The infant is maintaining saturation with minimal pres-

sures on synchronized intermittent positive pressure
ventilation, and hence extubation is planned. He has mini-
mal spontaneous respiratory efforts (respiratory rate
20 breaths/min) and develops cyanosis and bradycardia
within minutes of extubation, leading to reintubation.
However, there is no increase in pressure and oxygen
requirement. Bronchoscopy performed to rule out con-
genital malformations of the airway and lung has a normal
result. A trial of extubation is considered, which failed
twice in an interval of 48 hours.
At this stage, it is decided to again review the history and
clinical examination findings to ascertain the cause for recur-
rent extubation failure. Characteristic posture noted in the
upper limbs was adduction, internal rotation of arm with
pronation, and extension at elbow joint. This was suggestive
of Erb palsy. There is no movement of the upper limbs with
hypotonia, power of 1/5 (grading on the Medical Research
Council Scale for Muscle Strength), and absent biceps and Figure 3. Magnetic resonance imaging scan showing injury to spinal cord.
triceps jerk. On the contrary, posture, tone, power, and
reflexes are normal in the lower limbs. Also, the infant has
computed tomography of the spine reveal C3-C5 root avulsion
normal sensorium. Repeat chest radiography at this stage
injury leading to pseudomeningocele (Figs 3–5). In view of
reveals elevated bilateral diaphragms, more on the right side.
prolonged respiratory support, tracheostomy is planned.
In the background of difficult delivery, this correlates with
birth injury, leading to phrenic nerve injury and bilateral
diaphragmatic palsy. (Fig 2) Magnetic resonance imaging and

Figure 2. Radiograph showing elevated right hemidiaphragm (after

tracheostomy). Figure 4. Magnetic resonance imaging scan showing injury to spinal cord.

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Figure 5. Computed tomography scan showing cervical injury.
Diaphragmatic plication is done on the right side. He
undergoes ventilation for a period of approximately 45
days. During this course, he develops spontaneous
movements of the left upper limb, with the power
increased to 3/5, with no improvement on the right side.
The infant is weaned to continuous positive airway
pressure (CPAP) 5 days after surgery and then to room
air. He is discharged at 3 1/2 months of age. At the time of
discharge, he is active, alert, accepting feeds well, and
gaining weight with minimal distress.
DISCUSSION
Birth injuries are a diverse set of conditions occurring in a
neonate because of a traumatic event during the process of
delivery. (1) Diaphragmatic palsy resulting from phrenic
nerve injury is a relatively uncommon form of birth injury,
but nearly 80% to 90% of cases have associated brachial
plexus injury. (2) Isolated diaphragmatic palsy is uncom-
mon, with a prevalence of about 0.14 per 1,000 live births.
(3)
Apart from birth injury, other common etiologies include
iatrogenic (cardiothoracic surgery, invasive cannulation) and
neuromuscular disorders. Injury to the phrenic nerve
during cardiovascular surgery is the most common cause
followed by birth injury. (4)
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Injury to the phrenic nerve and spinal cord occurs
because of excessive traction placed on the spine during
delivery of the shoulder in a cephalic presentation and
delivery of the head in a breech presentation. (2) The risk
of injury is higher during the neonatal period because of
ligament laxity, weak musculature, and incomplete miner-
alization of the vertebrae. (3)
Diaphragmatic palsy following birth injury is most com-
monly unilateral (right>left). Bilateral involvement, which
occurs in less than 10% of cases, is seen in neonates with
severe birth injury. (5)(6) In the current case, the birth injury
was severe enough to cause bilateral diaphragmatic palsy.
Diaphragmatic palsy should be suspected when there
are recurrent failed extubation attempts in the back-
ground of a traumatic delivery, especially with breech
presentation. Affected neonates can have decreased chest
movement on the affected side, with corresponding
increased movement on the unaffected side and paradox-
ical chest movement. (7)
Because most of these neonates receive positive pres-
sure ventilation, clinical and radiologic findings can be
easily obscured, as in our case. Elevated hemidiaphragm,
which is usually seen in these cases, may not be present if
the neonate is receiving positive pressure ventilation. (2)
Ultrasonography is the preferred method because it is
safe, does not carry any risk of radiation exposure, and
can be done serially to assess the diaphragmatic function.
(8)
Management includes supportive care in the form of
supplemental oxygen, nasal CPAP, and mechanical ven-
tilation depending on the severity. CPAP has been
shown to be beneficial in some patients and a trial
should be given in every neonate because it avoids
intubation. (2)
Surgical intervention should be considered usually after 1
to 2 months of positive pressure ventilation when there is no
recovery. (2) Surgical plication of the affected diaphragm is
the commonly performed procedure and a satisfactory
response is seen in most cases. (9)
Phrenic nerve stimulation may help in making a decision
regarding surgery, with prolonged conduction latencies or
reduction in amplitude or absence of diaphragmatic action
potentials indicating poor chances of spontaneous recovery.
(10)
In the current case, the main presentation was recurrent
extubation failures with minimal ventilator settings and
normal sensorium. This infant also had bilateral Erb palsy,
which led us to evaluate for associated phrenic nerve injury
because of the spinal cord trauma leading to diaphragmatic
palsy.
Vol. 20 No. 11 N O V E M B E R 2 0 1 9 e665
 Lessons for the Clinician
• Diaphragmatic palsy should be considered in neonates in
cases of recurrent extubation failures, especially if it is
associated with a history of abnormal presentation or
traumatic delivery.
• All cases of Erb palsy with respiratory distress or ex-
tubation failures should be evaluated for associated
phrenic nerve injury.
• Clinical and radiologic signs of diaphragmatic palsy can be
missed in a neonate receiving positive pressure ventilation.
• Imaging of the spine is recommended to rule out other
associated injuries.
American Board of Pediatrics
Neonatal-Perinatal Content
Specification
• Know the clinical features and prognosis of birth injuries, such as
fractures, lacerations, and facial palsies.
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 Case 1: An Enigma of Recurrent Extubation Failure in a Neonate
Ayesha Romana, Indhuja Rajarathinam, Prathik Bandiya, Rajendra Shinde, Niranjan
Shivanna and Naveen Benakappa
NeoReviews 2019;20;e663
DOI: 10.1542/neo.20-11-e663

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 Case 1: An Enigma of Recurrent Extubation Failure in a Neonate
Ayesha Romana, Indhuja Rajarathinam, Prathik Bandiya, Rajendra Shinde, Niranjan
Shivanna and Naveen Benakappa
NeoReviews 2019;20;e663
DOI: 10.1542/neo.20-11-e663

The online version of this article, along with updated information and services, is
located on the World Wide Web at:
http://neoreviews.aappublications.org/content/20/11/e663

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