Chapter 31 Neonatal and Pediatric Respiratory Disorders.

Chapter 31Chapter 31

Neonatal and Pediatric Neonatal and Pediatric Respiratory DisordersRespiratory Disorders

Mosby items and derived items © 2009 by Mosby, Inc., an affiliate of Elsevi22er Inc. 2

Objectives Objectives

Discuss the clinical findings, radiographic Discuss the clinical findings, radiographic abnormalities, and treatment of patients with RDS.abnormalities, and treatment of patients with RDS.

Describe the clinical manifestations and treatment of Describe the clinical manifestations and treatment of patients with transient tachypnea of the newborn patients with transient tachypnea of the newborn (TTN).(TTN).

Describe the pathophysiology, presentation, and Describe the pathophysiology, presentation, and treatment of meconium aspiration syndrome.treatment of meconium aspiration syndrome.

Discuss the clinical signs and symptoms associated Discuss the clinical signs and symptoms associated with bronchopulmonary dysplasia and the with bronchopulmonary dysplasia and the approaches used to manage these infant.approaches used to manage these infant.


Objectives (cont.)Objectives (cont.)

Discuss the etiology and treatment of apnea of Discuss the etiology and treatment of apnea of prematurity.prematurity.

Describe the pathophysiology, diagnosis, and Describe the pathophysiology, diagnosis, and treatment of persistent pulmonary hypertension of the treatment of persistent pulmonary hypertension of the newborn (PPHN).newborn (PPHN).

Describe the pathophysiology, diagnosis, and Describe the pathophysiology, diagnosis, and treatment of congenital diaphragmatic hernia.treatment of congenital diaphragmatic hernia.

Describe the anatomic defects associated with Describe the anatomic defects associated with tetralogy of Fallot.tetralogy of Fallot.



Describe the clinical presentation of a Describe the clinical presentation of a ventricular septal defect. ventricular septal defect.

Define the epidemiologic factors associated Define the epidemiologic factors associated with increased risk of SIDS.with increased risk of SIDS.

Discuss the respiratory problems associated Discuss the respiratory problems associated with gastroesophageal reflux disease.with gastroesophageal reflux disease.

Describe the clinical findings commonly Describe the clinical findings commonly observed in patients with bronchiolitis.observed in patients with bronchiolitis.



Describe the clinical features and treatment Describe the clinical features and treatment of children epiglottitis. of children epiglottitis.

Describe the clinical manifestations and Describe the clinical manifestations and treatment of cystic fibrosis.treatment of cystic fibrosis.


Lung Parenchymal DiseaseLung Parenchymal Disease

Respiratory distress syndrome (RDS)Respiratory distress syndrome (RDS) Also called hyaline membrane diseaseAlso called hyaline membrane disease

Disorder of prematurity characterized byDisorder of prematurity characterized by Qualitative surfactant deficiencyQualitative surfactant deficiency

• Insufficient amounts released by type II cellsInsufficient amounts released by type II cells

• Poor quality so cannot decrease surface tensionPoor quality so cannot decrease surface tension

Decreased alveolar surface areaDecreased alveolar surface area Increased small airway complianceIncreased small airway compliance Patent ductus arteriosusPatent ductus arteriosus


Lung Parenchymal DiseaseLung Parenchymal Disease(cont.)(cont.)

Respiratory distress syndrome (cont.)Respiratory distress syndrome (cont.) Lung prematurity, and particularly the surfactant Lung prematurity, and particularly the surfactant

deficiency, sets up the following sequelae.deficiency, sets up the following sequelae. Alveolar instability and collapse, which leads to increased Alveolar instability and collapse, which leads to increased

WOBWOB Simultaneously increased surface tension draws fluid into Simultaneously increased surface tension draws fluid into

the alveolithe alveoli Combined, these cause impaired oxygenation with Combined, these cause impaired oxygenation with

hypoxemia and acidosis, which lead increased PVRhypoxemia and acidosis, which lead increased PVR Increases right-to-left shunting through the PDAIncreases right-to-left shunting through the PDA This worsens acidosis, hypoxemia, and surfactant This worsens acidosis, hypoxemia, and surfactant

production, which establishes a downward spiralproduction, which establishes a downward spiral



Lung Parenchymal DiseaseLung Parenchymal Disease (cont.) (cont.)

Respiratory distress syndrome (cont.)Respiratory distress syndrome (cont.) Clinical manifestationsClinical manifestations

Tachypnea occurs soon after birthTachypnea occurs soon after birth Followed by worsening retractions, paradoxical Followed by worsening retractions, paradoxical

breathing, and gruntingbreathing, and grunting• Nasal flaring may be seen.Nasal flaring may be seen.

Chest auscultation: fine inspiratory cracklesChest auscultation: fine inspiratory crackles Diagnosis is made from chest radiography.Diagnosis is made from chest radiography.

• Diffuse, hazy reticulogranular densities, air Diffuse, hazy reticulogranular densities, air bronchograms, and low lung volumes are typicalbronchograms, and low lung volumes are typical




Lung Parenchymal DiseaseLung Parenchymal Disease(cont.)(cont.)

Respiratory distress syndrome (cont.)Respiratory distress syndrome (cont.) TreatmentTreatment

Noninvasive CPAP (nasal prongs) preferredNoninvasive CPAP (nasal prongs) preferred• Trial with 4 to 6 cm HTrial with 4 to 6 cm H22O O

• Intubate and MV if PaIntubate and MV if PaOO22 does not improve, apnea or acidosis does not improve, apnea or acidosis occursoccurs

MV with PEEP: goals are to prevent alveolar collapse MV with PEEP: goals are to prevent alveolar collapse and provide adequate Vand provide adequate VAA

• Optimal PEEP provides lowest PaOptimal PEEP provides lowest PaCOCO22, highest Pa, highest PaOO22

• If high PaIf high PaCOCO22 persists, must increases f or PIP persists, must increases f or PIP

. .



Treatment of Respiratory distress syndrome Treatment of Respiratory distress syndrome (cont.)(cont.) Surfactant replacement instilled into trachea Surfactant replacement instilled into trachea

indicated for all RDS patientsindicated for all RDS patients High-frequency ventilation (HFV)High-frequency ventilation (HFV)


Lung Parenchymal Disease Lung Parenchymal Disease (cont.)(cont.)

Transient tachypnea of the newborn (TTN): type II RDSTransient tachypnea of the newborn (TTN): type II RDS May be most common newborn respiratory disorderMay be most common newborn respiratory disorder

Term babies with no specific predisposing factorsTerm babies with no specific predisposing factors

Probably occurs due to delayed clearance fetal lung fluidProbably occurs due to delayed clearance fetal lung fluid Birth canal squeeze normally accounts two-thirds Birth canal squeeze normally accounts two-thirds

clearanceclearance• Cesarean section avoids this squeeze Cesarean section avoids this squeeze

Lymphatics’ absorption accounts for final thirdLymphatics’ absorption accounts for final third• Immature lymphatics impairs absorptionImmature lymphatics impairs absorption



Transient tachypnea of the newborn (cont.)Transient tachypnea of the newborn (cont.) Clinical manifestationsClinical manifestations

No. 1 is tachypneaNo. 1 is tachypnea VVAA, pH, and Pa, pH, and PaCOCO22 are normal. Features of chest are normal. Features of chest

radiographradiograph• May look like pneumoniaMay look like pneumonia

• HyperinflationHyperinflation

• Pleural effusionsPleural effusions

• Perihilar streaking (lymphatic engorgement)Perihilar streaking (lymphatic engorgement)

. .



Transient tachypnea of the newborn (cont.)Transient tachypnea of the newborn (cont.) Treatment Treatment

Responds well to low FResponds well to low FIOIO22 with or without CPAP with or without CPAP

May use antibiotics, TTN hard to tell from May use antibiotics, TTN hard to tell from pneumoniapneumonia

Most have rapid response (24 to 48 hours)Most have rapid response (24 to 48 hours)



Meconium aspiration syndrome (MAS)Meconium aspiration syndrome (MAS) Disorder of term or near-term infants, associated with Disorder of term or near-term infants, associated with

perinatal depression and asphyxia perinatal depression and asphyxia

PathophysiologyPathophysiology Depression, asphyxia lead to passing of meconiumDepression, asphyxia lead to passing of meconium MAS involves three primary problems.MAS involves three primary problems.

• Pulmonary obstruction due to meconium pluggingPulmonary obstruction due to meconium plugging

• Lung damage due to chemical injuryLung damage due to chemical injury

• Persistent pulmonary hypertension (PPHN)Persistent pulmonary hypertension (PPHN)



Meconium aspiration syndrome (MAS)Meconium aspiration syndrome (MAS) Clinical manifestationsClinical manifestations

Gasping respirations, tachypnea, grunting, Gasping respirations, tachypnea, grunting, retractionsretractions

Chest radiographChest radiograph• Irregular pulmonary densities (atelectasis)Irregular pulmonary densities (atelectasis)• Hyperlucent areas (hyperinflation)Hyperlucent areas (hyperinflation)

ABGs: hypoxemia, mixed acidosis due to PPHNABGs: hypoxemia, mixed acidosis due to PPHN TreatmentTreatment

Immediate intubation and suctioningImmediate intubation and suctioning Mechanical ventilation Mechanical ventilation HFV, surfactant, and NO therapy may all be usedHFV, surfactant, and NO therapy may all be used





Bronchopulmonary dysplasia (BPD)Bronchopulmonary dysplasia (BPD) Pathophysiology: MultifactorialPathophysiology: Multifactorial

Atelectrauma is a result and cause of lung injury.Atelectrauma is a result and cause of lung injury. Volutrauma injures airways and lung parenchyma.Volutrauma injures airways and lung parenchyma. Above two synergistically increase lung injury, Above two synergistically increase lung injury,

necessitate oxygen therapy (leads to oxygen necessitate oxygen therapy (leads to oxygen toxicity)toxicity)

Immaturity, genetics, malnutrition, oxygen toxicity, Immaturity, genetics, malnutrition, oxygen toxicity, hypoxia, MV all contribute to BPD developmenthypoxia, MV all contribute to BPD development


Lung Parenchymal Disease Lung Parenchymal Disease (cont.) (cont.)

Bronchopulmonary dysplasia (cont.)Bronchopulmonary dysplasia (cont.) Clinical manifestationsClinical manifestations

Progressive respiratory distress at 2–3 weeks of Progressive respiratory distress at 2–3 weeks of lifelife• IF did not require OIF did not require O22 and MV before, will now and MV before, will now

Chest radiograph shows diffuse areas of Chest radiograph shows diffuse areas of atelectasis, emphysema, and fibrosis.atelectasis, emphysema, and fibrosis.

ABGs show varying hypoxemia and hypercapnia.ABGs show varying hypoxemia and hypercapnia. TreatmentTreatment

Prevention of atelectrauma and volutrauma is KEYPrevention of atelectrauma and volutrauma is KEY


Lung Parenchymal Disease Lung Parenchymal Disease (cont.) (cont.)

Bronchopulmonary dysplasia (cont.)Bronchopulmonary dysplasia (cont.) TreatmentTreatment

Establish optimal FRC in delivery roomEstablish optimal FRC in delivery room Early use of surfactantEarly use of surfactant Minimize lung damage: monitor carefully and use Minimize lung damage: monitor carefully and use

lowest oxygen and MV settings possiblelowest oxygen and MV settings possible Diuretics to treat pulmonary edemaDiuretics to treat pulmonary edema Antibiotics for pulmonary infectionsAntibiotics for pulmonary infections Chest physiotherapy for retained secretionsChest physiotherapy for retained secretions Bronchodilator therapy to decrease RBronchodilator therapy to decrease Rawaw Steroids used cautiously as create as many problems Steroids used cautiously as create as many problems

as solveas solve



Control of BreathingControl of Breathing

Apnea of prematurityApnea of prematurity Common in premature infantsCommon in premature infants

Apnea is abnormal if >15 seconds OR it causes cyanosis, Apnea is abnormal if >15 seconds OR it causes cyanosis, pallor, hypotonia, or bradycardiapallor, hypotonia, or bradycardia

Central apnea: no respiratory effort during apneaCentral apnea: no respiratory effort during apnea

Obstructive apnea: effort but no flowObstructive apnea: effort but no flow

Mixed apnea: combination of central and obstructive Mixed apnea: combination of central and obstructive apneaapnea


Control of Breathing Control of Breathing Apnea of prematurity (cont.)Apnea of prematurity (cont.) EtiologyEtiology

Immature chemo-control of respiratory driveImmature chemo-control of respiratory drive


Control of Breathing (cont.)Control of Breathing (cont.)


Pulmonary Vascular DiseasePulmonary Vascular Disease

Persistent pulmonary hypertension of the newborn (PPHN)Persistent pulmonary hypertension of the newborn (PPHN) Prolonged postpartum fetal circulation due to Prolonged postpartum fetal circulation due to ⇑⇑ PVR PVR

PathophysiologyPathophysiology Pulmonary blood flow is low due to right-to-left shunt Pulmonary blood flow is low due to right-to-left shunt

through a patent foramen ovale or ductus arteriosus.through a patent foramen ovale or ductus arteriosus.

Thee types of PPHNThee types of PPHN Vascular spasm: triggered by hypoxemia or painVascular spasm: triggered by hypoxemia or pain Increased muscle wall thickness: chronicIncreased muscle wall thickness: chronic Decreased cross-sectional area of vasculatureDecreased cross-sectional area of vasculature

• Lung hypoplasiaLung hypoplasia


Pulmonary Vascular Disease Pulmonary Vascular Disease (cont.)(cont.)

Persistent pulmonary hypertension of the Persistent pulmonary hypertension of the newborn (cont.)newborn (cont.)

Clinical manifestationsClinical manifestations Suspect when rapidly changing SpSuspect when rapidly changing SpOO22, hypoxemia , hypoxemia

worse than indicated on chest radiographworse than indicated on chest radiograph Detected by performing preductal and postductal Detected by performing preductal and postductal

SpSpOO22

• Preductal SpPreductal SpOO22 > 5% postductal Sp > 5% postductal SpOO22


Pulmonary Vascular Disease Pulmonary Vascular Disease (cont.)(cont.)

Persistent pulmonary hypertension of the Persistent pulmonary hypertension of the newborn (cont.)newborn (cont.)

Treatment Treatment Correct underlying problemCorrect underlying problem

• Hypoxemia with oxygen, surfactant for RDSHypoxemia with oxygen, surfactant for RDS

May require intubation, MV, and sedation May require intubation, MV, and sedation Often require: paralysis, HFV, worst cases ECMOOften require: paralysis, HFV, worst cases ECMO


Congenital Abnormalities Congenital Abnormalities Affecting RespirationAffecting Respiration

Congenital airways diseasesCongenital airways diseases Three mechanismsThree mechanisms

Internal obstructionInternal obstruction• Laryngomalacia, tracheomalacia, laryngeal websLaryngomalacia, tracheomalacia, laryngeal webs

• Stridor, gas-trapping, wheezing, accessory musclesStridor, gas-trapping, wheezing, accessory muscles

External obstruction (symptoms similar to internal)External obstruction (symptoms similar to internal)• Neck or thoracic mass, vascular ringsNeck or thoracic mass, vascular rings

Airway disruptions (TE fistula)Airway disruptions (TE fistula)• Five types, all manifest as difficulty swallowing, frothing at Five types, all manifest as difficulty swallowing, frothing at

mouth, and chokingmouth, and choking

• Often associated with other congenital abnormalitiesOften associated with other congenital abnormalities


Congenital Abnormalities Congenital Abnormalities Affecting Respiration (cont.)Affecting Respiration (cont.)

Lung malformationsLung malformations Most common cystic adenomatoid malformation: C-CAMMost common cystic adenomatoid malformation: C-CAM

Usually treat by surgical resection of affected lobeUsually treat by surgical resection of affected lobe Symptoms of volume loss, cardiopulmonary Symptoms of volume loss, cardiopulmonary

compromisecompromise

Congenital diaphragmatic herniaCongenital diaphragmatic hernia Manifests as severe respiratory distress, scaphoid Manifests as severe respiratory distress, scaphoid

abdomenabdomen

Combination of lung hypoplasia and abnormal Combination of lung hypoplasia and abnormal developmentdevelopment


Congenital Abnormalities Affecting Congenital Abnormalities Affecting Respiration (cont.)Respiration (cont.)

Congenital diaphragmatic hernia (cont.)Congenital diaphragmatic hernia (cont.) TreatmentTreatment

Initially intubation, paralysis, MV, continuous gastric sxInitially intubation, paralysis, MV, continuous gastric sx Repair delayed for PVR to fall due to adequate VRepair delayed for PVR to fall due to adequate VAA Severe cases may require HFV and ECMO.Severe cases may require HFV and ECMO.

Abdominal wall abnormalitiesAbdominal wall abnormalities Affect breathing as infants are abdominal breathersAffect breathing as infants are abdominal breathers If severe may require MVIf severe may require MV

Neuromuscular controlNeuromuscular control Spinal muscular atrophy, congenital myasthenia gravisSpinal muscular atrophy, congenital myasthenia gravis May require MV if severeMay require MV if severe


Congenital Heart DiseaseCongenital Heart Disease

Cyanotic congenital heart defectsCyanotic congenital heart defects Tetralogy of Fallot: comprised ofTetralogy of Fallot: comprised of

Pulmonary stenosis, VSD, dextroposition aorta, RVHPulmonary stenosis, VSD, dextroposition aorta, RVH Timing of surgical repair dependent on case Timing of surgical repair dependent on case

Transposition of the great arteries Transposition of the great arteries Manifests as moderate to severe cyanosis at birthManifests as moderate to severe cyanosis at birth Emergency atrial septostomy relieves distressEmergency atrial septostomy relieves distress Perform arterial switch operation at 2–3 weeks of lifePerform arterial switch operation at 2–3 weeks of life


Congenital Heart Disease (cont.)Congenital Heart Disease (cont.)

Acyanotic congenital heart defectsAcyanotic congenital heart defects Ventricular septal defect (VSD): most commonVentricular septal defect (VSD): most common

Results in left-to-right shunt and CHFResults in left-to-right shunt and CHF Appears 6–8 weeks as PVR fallsAppears 6–8 weeks as PVR falls

Atrial septal defect (ASD): typically of little importanceAtrial septal defect (ASD): typically of little importance Most common; incomplete closure of foramen ovaleMost common; incomplete closure of foramen ovale

Patent ductus arteriosus (PDA)Patent ductus arteriosus (PDA) Typically occurs in premature infantsTypically occurs in premature infants May act as a right-to-left or left-to-right shunt depending on May act as a right-to-left or left-to-right shunt depending on

SVR and PVRSVR and PVR



Pediatric Respiratory DisordersPediatric Respiratory Disorders

Sudden infant death syndrome (SIDS)Sudden infant death syndrome (SIDS) Leading cause of death in infants < 1 year of Leading cause of death in infants < 1 year of

ageage

Prevention of SIDS Prevention of SIDS Identification of infant at risk Identification of infant at risk Train family in apnea monitoring and CPR.Train family in apnea monitoring and CPR. Place patient in supine or side-lying position.Place patient in supine or side-lying position. Reduce soft objects in sleeping environment.Reduce soft objects in sleeping environment.


Pediatric Respiratory Disorders Pediatric Respiratory Disorders (cont.)(cont.)



Gastroesophageal reflux disease (GERD)Gastroesophageal reflux disease (GERD) Associated respiratory problems include Associated respiratory problems include

Reactive airways disease, aspiration pneumonia, Reactive airways disease, aspiration pneumonia, laryngospasm, stridor, chronic cough, choking, laryngospasm, stridor, chronic cough, choking, apneaapnea

Once diagnosed, medical and surgical Once diagnosed, medical and surgical managementmanagement



Bronchiolitis: caused by respiratory syncytial virus Bronchiolitis: caused by respiratory syncytial virus (RSV)(RSV)

Clinical manifestationsClinical manifestations Usually follows a URTIUsually follows a URTI Slight fever and cough worsen to dyspnea, tachypnea Slight fever and cough worsen to dyspnea, tachypnea Inspiratory and expiratory wheezing may developInspiratory and expiratory wheezing may develop Radiograph shows hyperinflation and consolidationRadiograph shows hyperinflation and consolidation

Prophylaxis: immunization recommended BPD Prophylaxis: immunization recommended BPD infantsinfants

Treatment: relief of airway obstruction and Treatment: relief of airway obstruction and hypoxemia, MV with prolonged expiration used in hypoxemia, MV with prolonged expiration used in most severe casesmost severe cases



Croup: also called laryngotracheobronchitisCroup: also called laryngotracheobronchitis Viral infection resulting in subglottic swelling: most Viral infection resulting in subglottic swelling: most

common cause of obstruction in 6-month- to 6-year-common cause of obstruction in 6-month- to 6-year-oldsolds

Clinical manifestationsClinical manifestations Follows 2–3 days of nasal congestions, fever, coughFollows 2–3 days of nasal congestions, fever, cough Progressive inspiratory and expiratory stridorProgressive inspiratory and expiratory stridor Barking cough is characteristicBarking cough is characteristic Progression to dyspnea, cyanosis, and exhaustionProgression to dyspnea, cyanosis, and exhaustion Chest radiograph shows classic “steeple sign”Chest radiograph shows classic “steeple sign”



Pediatric Respiratory DisordersPediatric Respiratory Disorders(cont.)(cont.)

Croup (cont.)Croup (cont.) TreatmentTreatment

Mild to moderate disease may be limited to cool Mild to moderate disease may be limited to cool mistmist

May require supplemental oxygenMay require supplemental oxygen Aerosolized racemic epinephrine and Aerosolized racemic epinephrine and

dexamethasone may decrease severity of dexamethasone may decrease severity of respiratory symptoms.respiratory symptoms.

Budesonide may reduce severity of symptoms.Budesonide may reduce severity of symptoms. Severe cases progressing despite therapy may Severe cases progressing despite therapy may

require intubation and mechanical ventilation.require intubation and mechanical ventilation.



Epiglottitis (cause Epiglottitis (cause H. influenzaeH. influenzae type B) type B) Life-threatening infection resulting in supraglottic swellingLife-threatening infection resulting in supraglottic swelling Clinical manifestationsClinical manifestations

High fever, sore throat, stridor, labored breathingHigh fever, sore throat, stridor, labored breathing NOT associated with barking coughNOT associated with barking cough May have difficulty swallowing and muffled voiceMay have difficulty swallowing and muffled voice Lateral neck radiograph shows “thumb sign”Lateral neck radiograph shows “thumb sign”

Treatment: proceed with great careTreatment: proceed with great care Elective intubation under general anesthesia, place on Elective intubation under general anesthesia, place on

CPAP with low PSV (3 cm HCPAP with low PSV (3 cm H22O)O)




Cystic fibrosisCystic fibrosis Most common lethal genetic disease in white Most common lethal genetic disease in white

AmericansAmericans Gene mutation affecting chloride movement, Gene mutation affecting chloride movement,

particularly in exocrine glandsparticularly in exocrine glands Clinical manifestations Clinical manifestations

Most severely affected organs are:Most severely affected organs are:• Sweat glands, pancreas, and lungsSweat glands, pancreas, and lungs

Skin is very salty: mother notes when kissing infantSkin is very salty: mother notes when kissing infant• Sweat chloride test is diagnosticSweat chloride test is diagnostic

Pancreatic insufficiency leads to malnutrition, diarrhea, Pancreatic insufficiency leads to malnutrition, diarrhea, and steatorrheaand steatorrhea



Cystic fibrosis (cont.)Cystic fibrosis (cont.) Clinical manifestations Clinical manifestations

Lung disease is leading cause of death. Lung disease is leading cause of death. Patients produce copious amounts of thick Patients produce copious amounts of thick

sputum. sputum. • Retained secretions lead to recurrent infections, Retained secretions lead to recurrent infections,

atelectasis, pneumonia, or lung abscesses. atelectasis, pneumonia, or lung abscesses.



Cystic fibrosis (cont.)Cystic fibrosis (cont.) TreatmentTreatment

Pancreatic enzyme supplementsPancreatic enzyme supplements Regular chest physiotherapy, strenuous exerciseRegular chest physiotherapy, strenuous exercise DNase, 7% saline preserve lung functionDNase, 7% saline preserve lung function Antibiotics are crucial to treat bronchiectatic flare-Antibiotics are crucial to treat bronchiectatic flare-

upsups May use antiinflammatory agents and May use antiinflammatory agents and

bronchodilatorsbronchodilators

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Chapter 31 Neonatal and Pediatric Respiratory Disorders.

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