differential diagnosis and management of respiratory … management of respiratory distress.pdf ·...

Differential Diagnosis and Management of Respiratory Distress Karen Wright, PhD, NNP-BC DNP NNP Program Director Rush University, Chicago, IL

The speaker has signed a disclosure form and indicated she has no significant financial interest or relationship with the companies or the manufacturer(s) of any commercial product and/or service that will be discussed as part of this presentation.

Session Summary This presentation will provide an overview of airway issues, diseases, mechanical, structural, obstructive, and iatrogenic causes of neonatal respiratory distress. The speaker will review the key characteristics, stabilization, and treatment options for the conditions discussed.

Session Objectives Upon completion of this presentation, the participant will be able to:

understand the phases of fetal lung disease;

discuss the physiology related to neonatal respiratory disorders;

identify management strategies and apply them to neonatal illness;

recognize respiratory emergencies and emergent management.

Test Questions

1. At the end of which stage is the lung considered viable?

a. Embryonic b. Pseudoglandular c. Canalicular d. Saccular e. Alveolar f. Vascular

2. What is the most common associated anomaly with TEF and EA?

a. Renal b. Cardiac c. Vertebral d. Limb e. CNS

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FANNP 27th National NNP Symposium: Clinical Update and Review

B11: Differential Diagnosis and Management of Respiratory Distress of 17

3. Which direction is blood shunted in babies with PPHN?

a. Right to left b. Left to right c. Bidirectional

4. You are called to evaluate an intubated 4-day old infant born at 25 weeks’ gestation. Oxygen saturation is 50% and the heart rate is 75 beats per minute and the baby is pale and cyanotic. The ETT has been suctioned with no occlusion. You bag by hand with a Pip of 30 without chest wall rise. What intervention will you do next?

d. Administer an IV caffeine bolus e. Continue to hand bag with a higher pressure f. Obtain an emergent chest x-ray g. Remove the ETT and bag-mask ventilate h. Start chest compressions

5. Which of the following is true about self-inflating bags?

a. It cannot reliably delivery free flow 100% oxygen b. It does not have a safety pop-off valve c.. It does not fill spontaneously after it is squeezed d. It requires a gas source to inflate e. It requires a tight seal to maintain inflation of the bag

References American Academy of Pediatrics (2016). Textbook of neonatal resuscitation (7th ed.). American Academy of

Pediatrics and American Heart Association.

Brodsdy, D. & Martin, C. (2015). Neonatology review: Q & A (3rd ed.). Lulu Press, Inc.

Finer, N. & Leone, T. (2009). Oxygen saturation monitoring for the preterm infant: The evidence basis for current practice. Pediatric Research, 65, 375.

Gomella, T. (2004). Neonatology: Management, procedures, on-call problems, diseases, and drugs (7th ed.). New York: McGraw-Hill.

Hooper, S.B., Te Pas, A. B. & Kitchen, M. J. (2016). Respiratory transition in the newborn: A three-phase process. Archives of Disease in Childhood, Fetal and Neonatal Edition, 101, F266.

Jain, L. & Eaton, D. C. (2006). Physiology of fetal lung fluid clearance and the effect of labor. Seminars in Perinatology, 30(1), 34-43. doi: 10.1053/j.semperi.2006.01.006

Martin, R., Fanaroff, A. & Walsch, M. (2015). Neonatal-perinatal medicine (10th ed). Philadelphia: Elsevier Saunders.

Machado L., Fiori H., Baldisserotto M., et al. (2011). Surfactant deficiency in transient tachypnea of the newborn. Journal of Pediatrics, 159, 750.

Polin, R. & Yoder, M. (2015). Workbook in practical neonatology. Philadelphia: Elsevier Saunders.

Siew, M., Te Pas, A., Wallace, M., et al.1985 (2009). Positive end-expiratory pressure enhances development of a functional residual capacity in preterm rabbits ventilated from birth. Journal of Applied Physiology, 106, 1487.



Studying

Differential Diagnosis and Management of Respiratory Distress

Karen Wright PhD, NNP‐BC

Rush University Chicago, IL

• Part 1 Developmental Disorders of the Respiratory System

• Part 2 Respiratory Diseases

• Part 3 Basic Management of the Airway

• Part 4 Respiratory Emergencies

Part 1 Lung Development and Maturation

• Embryonic Period

• Pseudoglandular Stage

• Canalicular Stage

• Saccular and Alveolar Stages

What is a differential diagnosis?



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Differential diagnosis

• Think of problems by system (ex. respiratory, cardia)

• View babies by system symptoms • A diagnosis is what is known or suspected enough to consider for treatment, or has been diagnosed (x‐ray – RDS, ABG – acidosis)

• Diagnosis leads to management • Neonates often have concurrent diagnoses (RDS & Sepsis)

• Classic example – Respiratory versus Cardiac

Gestational Phases of Lung Development

• Beginning – Embryologic and Pseudoglandular

• Middle – Canalicular and Saccular

‐Capable of gas exchange

‐ Pneumatocytes (Type I & II)

‐ Capillary beds

• Later – Alveolar and microvascular

Stages of Lung Development (Kajekar et. al)

Acinus

Branching Development of the Human Lung

Embryonic ............... .· .·

Pseudoglandular

Lung bud differentiation

Trachea and bronchi

Pulmonary vein and artery

Conducting airways

Terminal bronchioles

Canalicular ''' ''' ' ' '

Saccular '

Alveoli saccules

Extra-celfu/ar matrix

Neural network

Alveolar

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-----,''

Immature neural networl<s

Pre-acinar blood vessels Primitive alveoli

I, Type If 'I I

..._ _, ' '

Expansion of gas exchange area, nerves and capil/aries

Continued cellular proliferation

Lung growth and expansion

4-7weeks 7-17 weeks 17-26 weeks 27-36 weeks

In utero

36 weeks- 2 years

Birth

Postnatal

18years

Embryonic Lung Development

Pharynx Esophagus

Trachea

....

Embryonic Phase • Begins with groove in ventral lower pharynx

• 2 bud form and develop asymmetrically Laryngotracheal

tube

------.T.ra.chea Bronchial bud • Subdivides into 2 bronchi

Splanchnic mesoderm

Upper lobe

Middle lobe

Lower lobe

Beginning of 4th week

8 weeks

Tracheal buds Tracheal buds \

End of

4 weeks ._

bifurcates

Bronchial buds develop

opens tax ens

• Disorders during this phase:

Atresias (laryngeal, esophageal, tracheal_

Bronchogenic cysts

TEF

Pulmonary sequestration

Moore, Human Development



Pseudoglandular (5‐17 weeks) • Disorders during this time

– Renal agenesis (pulmonary hypoplasia)

– CCAM

– Pulmonary lymphangiectasis

– CDH

– Tracheomalacia/bronchomalacia

Canalicular (16‐26 weeks) • Renal dysplasia and pulmonary hypoplasia

• Alveolar capillary dysplasia

• Surfactant deficiency

Saccular (24‐38 weeks) • Oligohydramnios and pulmonary hypoplasia

• Alveolar capillary dysplasia


Alveolar (36 weeks+) • Lobar emphysema

• Pulmonary hypertension


Vascular 36 + weeks • Derived from 6th aortic arch during development

Phases of Lung Development

10 12 14 18 2D 22 26 28 30 32 34



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QUESTION Stages of Lung Development (Kajekar et. al)

Acinus

Embryonic ............... .· .·

At the end of which stage is the lung considered viable?

A. Embryonic

B. Pseudoglandular

C. Canalicular

Lung bud differentiation

Trachea and bronchi

Pulmonary vein and artery

Conducting airways

Terminal bronchioles

Pseudoglandular

Canalicular ''' ''' ' ' '

Saccular '

Alveoli saccules

Extra-celfu/ar matrix

Neural network

----- Alveolar

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' ,''

D. Saccular

E. Alveolar

F. Vascular

Immature neural networl<s

Pre-acinar blood vessels Primitive alveoli

I, Type If 'I I

..._ _, ' '

Expansion of gas exchange area, nerves and capil/aries

Continued cellular proliferation

Lung growth and expansion

4-7weeks 7-17 weeks 17-26 weeks 27-36 weeks

In utero

36 weeks- 2 years

Birth

Postnatal

18years

QUESTION

At the end of which stage is the lung considered viable?

A. Embryonic

B. Pseudoglandular

C. Canalicular

D. Saccular

E. Alveolar

F. Vascular

How will you ever remember the stages of lung development?

Each Person Can Study Alone vigorously

From Lucki Jain • Each Embryonic (5 weeks)

• Person Pseudoglandular (15 weeks)

• Can Canalicular (25 weeks)

• Study Saccular (35 weeks)

• Alone Alveolar (3‐5 years)

• Vigorously Vascular (3‐5 years)

Case Study ‐A G2P1→2 28 year old woman gives birth to a 38 week baby boy weighing 3,200g by NSVD; prenatal history is uncomplicated. Fundal height consistently agreed with dates throughout the pregnancy. ‐A routine post‐date non‐stress test one week before delivery was reactive. An abdominal ultrasound obtained at the same time revealed an amniotic fluid index of 26. At delivery, the infant was vigorous and had Apgar scores of 9 at one minute and 9 at five minutes. Initial physical examination at one hour of life was remarkable only for a moderate increase in white oral secretions, which cleared with suctioning. The patient passed a meconium stool in the first six hours of life and tolerated his first feeding with minimal coughing and sneezing.



At approximately 8 hours of life, the infant was noted to have increased frothing and coughing after breastfeeding, and his abdomen was mildly dilated. Crackles were heard all throughout the lung fields. Vitals signs were all within normal limits. A catheter was gently passed into the esophagus and met resistance.

What is the diagnosis?

What is the significance of the AFI? • Fetus swallows 100/mls/kg of amniotic fluid every day

• Amniotic fluid is produced by fetal kidneys

Based on the history, you suspect this baby has:

A. Congenital Diaphragmatic Hernia

B. Respiratory Distress Syndrome

C. Tracheoesophageal fistula

Bonus – During which phase on development did this disorder originate?

Tracheoesophageal Fistula • Tracheoesophageal Fistula

• A Tracheoesophageal Fistula (TEF) is an abnormal passage between the trachea and esophagus

• 1: 3000

• Males

• Usually associated with Esophageal Atresia

• Incomplete division of the foregut during 4th week of gestation

• Septum between trachea and esophagus is defective (

What diagnostic study would you order?

A. PA and lateral chest X‐ray & KUB

B. Upper GI with Contrast

C. Abdominal CT



After ABCs, what is your priority for managing this infant?

A. Septic work‐up

B. Prevent aspiration

C. Complete physical exam

Respiratory Disorders of Embryonic Phase

What is the most common associated anomaly with TEF and EA?

A. Renal B. Cardiac C. Vertebral D. Limb

E. CNS

• Atresias (laryngeal, esophageal, tracheal)

• Bronchogenic cysts

• TEF

• Pulmonary agenesis

Pulmonary Hypoplasia

• Maternal history • Most common is secondary and due to

Restrictions is fetal breathing OR Restrictions in lung growth

***Usually vascular in origin • Due to deficient development of the lung parenchyma→↓number of distal airways, alveoli, pulmonary vessels

• Primary – caused by intrinsic lung development failure • Secondary – space occupying lesions compressing

lungs/preventing normal growth

What are some causes of secondary lung hypoplasia?

• Cardiomegaly

• Abnormal diaphragmatic activity (CNS or PNS)

• Congenital Diaphragmatic Hernia



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Disorders of Pseudoglandular Period

• Renal Agenesis

• CCAM

• CDH

• Tracheomalacia/bronchomalacia ;,:..r="ln•on

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Congenital Diaphragmatic Hernia • Developmental defect during diaphragm formation

• Allows herniation of abdominal contents into thoracic cavity

• Can be isolated or associated with other anomalies

• Pulmonary insufficiency and pulmonary hypertension secondary to pulmonary hypoplasia

Management of CDH • Delivery in a proficient center • Intra‐disciplinary team with a coordinated approach

• Lower PIP/gentler ventilation/permissive • HFOV • ECMO • Delayed surgical repair • Sometimes used by not yet proven beneficial: Surfactant, iNO

• Associated with sensorineural hearing loss

Martin, Fanaroff, Walsch, 2015

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heart and to the rig

------ Liver part; up into ch

Diaphragr hernia



CDH Repair

Lu

You are called to the delivery room to evaluate a full‐ term male infant who has just been born by virginal

birth. You observe that the infant is in severe respiratory distress without breath sounds on the left

side. The infant’s abdomen is scaphoid in appearance.

Sutured diaphragm

What is the most appropriate initial management of this infant? A. Insert a chest tube to evaluate air from the pleural

space B. Intubate the infant to minimize inflation pressure C. Place an umbilical venous line and start PGE1

D. Provide bag‐mask ventilation to re‐inflate the collapsed lung

Acquired Pulmonary Diseases • MAS

• Neonatal Pneumonia

• TTN

• Aspiration Syndromes

• Pulmonary Air Leak Syndromes

• Rib Cage Abnormalities

• Phrenic Nerve Injury

More Respiratory Disorders of Development

• Canalicular Phase (16‐26 weeks) ‐Surfactant Deficiency

• Saccular Phase (24‐38 weeks) ‐ Oligohydramnios ‐ Surfactant deficiency

• Alveolar Phase (36+ weeks) ‐ Lobar emphysema ‐ Surfactant deficiency

• Vascular Phase

Part 2 Respiratory Diseases • Meconium Aspiration

• Transient Tachypnea

• Pneumonia

• Respiratory Distress Syndrome

• PPHN



Meconium Aspiration Syndrome • Population

• Pathophysiology

• Clinical Presentation

• Differential Diagnoses

• Diagnostics

• Management

• Pharmacology

• Future trends

Transient Tachypnea • Population

• Pathophysiology



• Diagnostics

• Management

• Pharmacology

• Future trends

Neonatal Pneumonia/EOS • Population

• Pathophysiology



• Diagnostics

• Management

• Pharmacology

• Future trends



Respiratory Distress Syndrome • Population

• Pathophysiology



• Diagnostics

• Management

• Pharmacology

• Future trends

Lung maturation • Throughout all of the phases, lungs are in various stages of maturation

– At 24 weeks airways are completed through terminal bronchioles, gas exchange units are rudimentary

‐ At 24 weeks the alveolar membrane enables gas exchange

‐ Surface areas are expanding ‐ Capillary network is expanding ‐ True alveoli by 36 weeks

Physical Mediators of Lung Development

1. Lung Fluid – helps lungs stretch and grow

2. Fetal Breathing Movements (inspired by?)

3. Peristaltic Airway Contractions

4. Vitamin A Deficiency

5. Glucocorticoids, thyroid, retinoic acid

Back to RDS – What is the Hallmark Pathophysiology? Surfactant

• Composed of highly organized lipids an surfactant proteins

• Preterm lung is low in mature surfactant

• Molecules are hydrophobic and hydrophyllic (opposed at both ends)

• Function of surfactant is to

REDUCES SURFACE TENSION AT THE AIR FLUID INTERFACE and increases compliance of the lung and reduces the work of breathing



Surfactant – they will ask you this

Reduces surface tension at the air fluid interface and increases compliance of the lung and thereby reduces the work of breathing

Which of the following is true about surfactant deficient lungs?

A. They are stiff and non‐compliant

B. Result in ↑ WOB

C. Result in atelectasis and low lung volumes

D. Alveoli is filled with exudate

E. Gas diffusion is blocked

F. Results in hypoxia, ↑CO2, mixed acidosis

Types of Surfactant • Synthetic – ALEC, Exosurf

• Extracted – Survanta (bovine)

Curosurf (porcine)

Infasurf (calf)

Alveofact (bovide)

Venticute

Surfaxin

Actions of Surfactant • Acutely improves lung function (what does this mean to you as an NNP?)

• Surfactant substrate makes better lung surfactant

• Has a prolonged effect

Surfactant demonstration More about surfactant

• You need a large surface area to allow for gas exchange (How will we accomplish this? – hint is rhymes with creep)

• Surfactant decreases surface tension…………

• Type II pneumatocytes produce surfactant but also are protective



PPHN Which direction is blood shunted in babies with PPHN?

A. Right to left

B. Left to right

C. Bidirectional

PPHN Great website for more details about lung development

https://embryology.med.unsw.edu.au/embryolo gy/index.php/Respiratory_System_Development

• Hemodynamics

• Risk Factors

• Diagnosis

• Management

• Pharmacology

Risk Factors • CDH

• MAS

• Asphyxia


• Pneumonia

• Idiopathic

Management • Delivery room

• Support

• Diagnosis

• PPHN versus cardiac disease



Management Strategies • Oxygen

• Mechanical ventilation

• ECMO

• Medications

– iNO

‐ Surfactant

Newer Medications • Sidenafil

• Steroids

• Adenosine

• Milrinone

Other Respiratory Diseases • BPD

• Interstitial lung disease

• Choanal atresia

• Laryngomalacia

• Tracheobronchomalacia

• Tracheal stenosis

• Vascular airway abnormalities

Part 3 Basic Respiratory Management

• Primary and secondary apnea

• Oxygen

• Properties

• NRP recommendations

• Pulse oximetry

• Continuum of respiratory care

• Suctioning

CPAP • Physiology of PEEP

• Alveolar Recruitment

• Improving oxygenation

Part 4 Respiratory Emergencies • Air Leak

• Infections

• Congenital Diaphragmatic Hernia

• Pierre Robin Syndrome

• Abdominal Distention



Resuscitation • PLEASE STUDY NRP

• Understand the foundational underpinning of ventilation

• PPV as a non‐invasive methodology

• Primary apnea is a retrospective concept

• Naloxone is not an emergency drug

Intubation • Approach to intubation

• Indications for intubation

• Noninvasive ventilation

• Safety concerns

• Technique

Demonstration This is my trick for success when you are first learning the difficult skill of intubation

Transillumination



Needle Aspiration • Indications

• Landmarks and technique

Extrapulmonary Causes of Respiratory Distress

Neuromuscular – CNS, asphyxia, hemorrhage, malformations, drugs, birth injury, muscular dystrophies Obstructive – thoracic dystrophies, choanal atresias,tracheal stenosis, blocked ETT

Diaphragmatic Disorders – abdominal distention, eventrations Hematologic – polycythemia, anemia Metabolic – metabolic acidosis, hypoglycemia Cardiovascular – ↑pulmonary blood flow, ↓pulmonary blood flow, cardiomegaly Miscellaneous – sepsis, pain, hypothermia, hyperthermia

Martin, Fanarhoff, Walsch, 2015



differential diagnosis and management of respiratory … management of respiratory distress.pdf ·...

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