B11. Differential Diagnosis and Management of Respiratory Distress. Session Summary. Session Objectives. Test Questions

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1 B11 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 B11: Differential Diagnosis and Management of Respiratory Distress Page 1 of 17

2 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 (7 th ed.). American Academy of Pediatrics and American Heart Association. Brodsdy, D. & Martin, C. (2015). Neonatology review: Q & A (3 rd 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), doi: /j.semperi Martin, R., Fanaroff, A. & Walsch, M. (2015). Neonatal-perinatal medicine (10 th 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, B11: Differential Diagnosis and Management of Respiratory Distress Page 2 of 17

Respiratory Emergencies Part 1 Lung Development and Maturation Embryonic Period Pseudoglandular Stage Canalicular Stage Saccular

3 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? B11: Differential Diagnosis and Management of Respiratory Distress Page 3 of 17

management Neonates often have concurrent diagnoses (RDS & Sepsis) Classic example Respiratory versus Cardiac Gestational Phases of Lung Development Beginning Embryologic and Pseudoglandular Middle

4 0 FANNP 27th National NNP Symposium: Clinical Update and Review 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) Embryonic... Pseudoglandular.. Acinus Branching Development of the Human Lung Lung bud differentiation Trachea and bronchi Pulmonary vein and artery Conducting airways Terminal bronchioles Immature neural networl<s Pre-acinar blood vessels Primitive alveoli Canalicular ' ''''' ' ' ' Saccular ' Alveoli saccules Extra-celfu/ar matrix Alveolar Neural network Expansion of gas exchange area, nerves and capil/aries 0, ,'''' I, Type If 'I 4-7weeks 7-17 weeks weeks weeks In utero I..., ' ' t 36 weeks- 2 years Birth Continued cellular proliferation Lung growth and expansion 18years Postnatal Embryonic Lung Development Embryonic Phase Pharynx Laryngotracheal tube Splanchnic Tracheal buds mesoderm Beginning of 4th week Upper lobe Middle lobe Lower lobe 8 weeks... Esophagus Trachea T.ra.chea Bronchial bud Tracheal buds \ End of 4 weeks._ bifurcates Bronchial buds develop Begins with groove in ventral lower pharynx 2 bud form and develop asymmetrically Subdivides into 2 bronchi Disorders during this phase: Atresias (laryngeal, esophageal, tracheal_ Bronchogenic cysts TEF Pulmonary sequestration opens tax ens Moore, Human Development B11: Differential Diagnosis and Management of Respiratory Distress Page 4 of 17

5 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 Surfactant deficiency Alveolar (36 weeks+) Lobar emphysema Pulmonary hypertension Surfactant deficiency Vascular 36 + weeks Phases of Lung Development Derived from 6 th aortic arch during development D B11: Differential Diagnosis and Management of Respiratory Distress Page 5 of 17

0 FANNP 27th National NNP Symposium: Clinical Update and Review QUESTION At the end of which stage is the lung considered viable? A. Embryonic B. Pseudoglandular C. Canalicular D. Saccular E.

6 0 FANNP 27th National NNP Symposium: Clinical Update and Review QUESTION At the end of which stage is the lung considered viable? A. Embryonic B. Pseudoglandular C. Canalicular D. Saccular E. Alveolar F. Vascular Stages of Lung Development (Kajekar et. al) Embryonic Pseudoglandular t... Canalicular ' '' Lung bud differentiation ''' Trachea and bronchi ' '' Pulmonary vein and artery Saccular ' 0, 0 Alveoli saccules ',' Conducting airways Extra-celfu/ar matrix Alveolar Terminal bronchioles Neural network Expansion of gas exchange area, Immature neural networl<s nerves and capil/aries Pre-acinar blood vessels Primitive alveoli I, Type If 'I I Continued cellular proliferation..., ' ' Lung growth and expansion 4-7weeks 7-17 weeks weeks weeks In utero 36 weeks- 2 years Birth Postnatal.. Acinus 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 G2P 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. B11: Differential Diagnosis and Management of Respiratory Distress Page 6 of 17

At approximately 8 hours of life, the infant was noted to have increased frothing and coughing after breastfeeding, and his abdomen was mildly dilated.

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.

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

7 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 4 th 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 B11: Differential Diagnosis and Management of Respiratory Distress Page 7 of 17

8 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 B11: Differential Diagnosis and Management of Respiratory Distress Page 8 of 17

POTTERS SYN ROW e CorseouQF"ces of" Reno sor tt r = due- ge'""lesis Disorders of Pseudoglandular Period

.r="ln on osu,._ -rnor-,...o plo::sl"o :::e_ 10 I i:jea 1- -t r"q r'ft rc:,._p.-.:xtor '-' r r c -e.

of abdominal contents into thoracic cavity Can be isolated or associated with other anomalies Pulmonary

proficient center Intra disciplinary team with a coordinated approach Lower PIP/gentler

Surfactant, ino Associated with sensorineural hearing loss Martin, Fanaroff, Walsch, 2015 It:; I l vi IC.

9 POTTERS SYN ROW e CorseouQF"ces of" Reno sor tt r = due- ge'""lesis Disorders of Pseudoglandular Period RE"-AL "'.::..CE:N:...SIS U IRC:.IE I I Renal Agenesis CCAM CDH Tracheomalacia/bronchomalacia ;,:..r="ln on osu,._ -rnor-,...o plo::sl"o :::e_ 10 I i:jea 1- -t r"q r'ft rc:,._p.-.:xtor '-' r r c -e..,c't' 1 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 It:; I l vi IC..:)t, heart and to the rig Liver part; up into ch Diaphragr hernia B11: Differential Diagnosis and Management of Respiratory Distress Page 9 of 17

Lu CDH Repair You are called to the delivery room to evaluate a fullterm male infant who has just been born by virginal birth.

The infant s abdomen is scaphoid in appearance. Sutured diaphragm What is the most appropriate initial management of this infant? A.

Place an umbilical venous line and start PGE1 D.

Pulmonary Air Leak Syndromes Rib Cage Abnormalities Phrenic Nerve Injury More Respiratory Disorders of Development Canalicular Phase (16 26 weeks)

10 Lu CDH Repair You are called to the delivery room to evaluate a fullterm 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 B11: Differential Diagnosis and Management of Respiratory Distress Page 10 of 17

Meconium Aspiration Syndrome Population Pathophysiology Clinical Presentation

Tachypnea Population Pathophysiology Clinical Presentation Differential Diagnoses

11 Meconium Aspiration Syndrome Population Pathophysiology Clinical Presentation Differential Diagnoses Diagnostics Management Pharmacology Future trends Transient Tachypnea Population Pathophysiology Clinical Presentation Differential Diagnoses Diagnostics Management Pharmacology Future trends Neonatal Pneumonia/EOS Population Pathophysiology Clinical Presentation Differential Diagnoses Diagnostics Management Pharmacology Future trends B11: Differential Diagnosis and Management of Respiratory Distress Page 11 of 17

Respiratory Distress Syndrome Population Pathophysiology Clinical Presentation Differential Diagnoses Diagnostics Management Pharmacology Future trends Lung maturation Throughout all of the phases,

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.

12 Respiratory Distress Syndrome Population Pathophysiology Clinical Presentation Differential Diagnoses 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 B11: Differential Diagnosis and Management of Respiratory Distress Page 12 of 17

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

13 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 B11: Differential Diagnosis and Management of Respiratory Distress Page 13 of 17

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.

14 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 gy/index.php/respiratory_system_development Hemodynamics Risk Factors Diagnosis Management Pharmacology CDH MAS Asphyxia Surfactant deficiency Pneumonia Idiopathic Risk Factors Management Delivery room Support Diagnosis PPHN versus cardiac disease B11: Differential Diagnosis and Management of Respiratory Distress Page 14 of 17

Management Strategies Oxygen Mechanical ventilation ECMO Medications ino Surfactant Sidenafil Steroids Adenosine Milrinone Newer Medications Other Respiratory Diseases BPD Interstitial lung disease

15 Management Strategies Oxygen Mechanical ventilation ECMO Medications ino Surfactant Sidenafil Steroids Adenosine Milrinone Newer Medications 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 Physiology of PEEP Alveolar Recruitment Improving oxygenation CPAP Part 4 Respiratory Emergencies Air Leak Infections Congenital Diaphragmatic Hernia Pierre Robin Syndrome Abdominal Distention B11: Differential Diagnosis and Management of Respiratory Distress Page 15 of 17

PLEASE STUDY NRP Resuscitation Understand the foundational underpinning of

concept Naloxone is not an emergency drug Intubation Approach to intubation

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

16 PLEASE STUDY NRP Resuscitation 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 B11: Differential Diagnosis and Management of Respiratory Distress Page 16 of 17

Indications Landmarks and technique Needle Aspiration Extrapulmonary Causes of Respiratory Distress Neuromuscular CNS, asphyxia, hemorrhage, malformations, drugs, birth injury, muscular dystrophies

17 Indications Landmarks and technique Needle Aspiration 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 B11: Differential Diagnosis and Management of Respiratory Distress Page 17 of 17

Lecture 21Development of respiratory system Dr. Rehan Asad At the end of session students should able to Describe formation of lung buds Describe

Lecture 21Development of respiratory system Dr. Rehan Asad At the end of session students should able to Describe formation of lung buds Describe development of larynx, trachea and bronchi. Describe the