Andrea Kline Tilford Phd, CPNP‐AC/PC, FCCM

Acute Care Pediatric Nurse Practitioner Review Course 2020

Andrea Kline Tilford PhD, CPNP‐AC/PC, FCCM

C.S. Mott Children’s Hospital Ann Arbor, Michigan

• I have no financial relationships to disclose • I will not discuss investigational drug use

• Discuss general principles of pediatric respiratory physiology • Discuss the presentation and evaluation of common pediatric respiratory diseases • Identify appropriate management strategies for common pediatric respiratory diseases

©202 0 Basic Anatomy • Upper Airway • Supraglottic (nose, nasopharynx, epiglottis) • Glottis (vocal cords, subglottic area, cervical trachea) • Humidifies inhaled gases • Warms inhaled gas • Site of most resistance to airflow • Conducting airways (dead space) • Lower airways • Thoracic trachea, bronchi, bronchioles and alveoli (gas exchange)

• Pediatrics • Small mouth • Large tongue • In relation to mandible • Floppy epiglottis (infants) • Large occiput • Infants are obligate nose breathers (until ~ 6 months of age) • Cricoid ring narrowest portion of airway in infants and young children

• Bifurcates into right and left bronchus • RIGHT side generally more straight and more likely to be site of aspiration

www.med.umich.edu ©202 0 Alveoli

• Continue to multiply until ~ 8 years of age

Covered in capillaries

Site of gas exchange

oac.med.jhmi.edu ©202 0 Basic Physiology

• Goal of respiration = Oxygen in and carbon dioxide out • Oxygen ʻinʼ • For cell use • Carbon dioxide ʻoutʼ • Produced by cells

• Inhalation • Active; requires contraction of several muscles (e.g. diaphragm, intercostals) • Exhalation • Passive • Relaxation of intercostals and diaphragm, return of rib cage, diaphragm, and sternum to resting position, increases pressure in lungs and air is exhaled **PEARL: Some conditions, such as status asthmaticus, interfere with passive exhalation. Physical exam demonstrates forced and prolonged exhalation phase.

• Under neural and chemical control • Hypoventilation should always raise concern for neuronal anomaly (e.g. drug effect, underlying intracranial process, others) • Hyperventilation often caused by conditions outside the lung (metabolic acidosis, neurologic process, anxiety)

• Definition • Acute inflammatory disease of the lower respiratory tract • Etiology • Many different viral agents • Respiratory syncytial virus (RSV) and influenza account for most cases when a specific agent can be identified • Other common agents: Rhinovirus, adenovirus, parainfluenza, human metapneumovirus • Incidence • Peaks in winter months, though common fall – spring • Risk Factors for severe disease • Prematurity, cardiopulmonary disease, infants < 6 months of age, immunodeficiency

www.nlm.nih.gov ©202 0 Bronchiolitis

• Pathophysiology • Inflammatory disease of lower respiratory tract • Also, a disease of lung parenchyma • Clinical Manifestations • Rhinorrhea, congestion • Tachypnea, increased work of breathing • Hypoxia Flat diaphragms • Fever • Wheezing; prolonged exhalation • *Typically begins as upper respiratory infection that spreads to lower airways in first few days of illness • Diagnostic Evaluation • History and physical; diagnostic in most cases • Chest radiograph (Commonly demonstrates hyperexpansion, patchy atelectasis, peribronchial thickening) • Viral studies are supportive (RSV, influenza, etc) • Pulse oximetry

www.surgery.usc.edu Bronchiolitis Chest Xray

Peribronchial Bronchial wall thickening thickening

www.learningradiology.com

• Management • Supportive care • Oxygen • Additional respiratory maneuvers, if indicated • Routine use of bronchodilators, racemic epinephrine, and corticosteroids are NOT recommended; AAP guidelines • Prevention • Hand washing • Palivizumab®, as indicated

http://pediatrics.aappublications.org/content/early/2014/10/21/peds.2014-2742

• Definition • Infection involving subglottic airway, larynx, trachea, and bronchi • Etiology • Most commonly caused by parainfluenza type 1 and 2 • Less commonly caused by respiratory syncytial virus (RSV), adenovirus, influenza types A and B, parainfluenza type 3 • Infrequently caused by Mycoplasma pneumoniae • Incidence • Most commonly affects children 3 months ‐ 5 years of age • Some children are at risk for recurrent episodes • Northern hemisphere; most common between October and March

• Pathophysiology • Mucosal airway edema from infectious etiology • Subsequent epithelial necrosis • Decreased airway diameter and increased resistance to airflow • Clinical Manifestations • ʻBarkyʼcough • Stridor • Hoarseness • Increased work of breathing • Wheezing/prolonged expiratory phase • Frequently begins 12‐ 48 hours after non‐specific upper respiratory tract symptoms • Typically, symptoms are worse at night

• Evaluation • Westley Croup score or other scoring tool may be used • Stridor, retractions, air entry, cyanosis, level of consciousness • Diagnosis • Generally based on history and examination • Viral studies may be sent to identify the pathogen • Consider lateral neck films (e.g. “Steeple sign”) • Consider chest radiograph; may be obtained to evaluate for superimposed bacterial infection and/or narrowing of subglottic area

•Management • Humidified or cool mist air/gas • Steroids • Dexamethasone 0.6 mg/kg IV/IM • Oxygen and respiratory maneuvers, as needed • Consider aerosolized racemic epinephrine • Consider heliox (light gas, improves laminar flow) • Intravenous fluids, if dehydrated • Otolaryngology consult if no improvement with standard therapy

Definition • Asthma • Chronic reversible disorder resulting in inflammation, bronchoconstriction, airway hyperresponsiveness • Characterized by episodes of cough, wheeze, dyspnea, chest tightness • Status Asthmaticus • Progressively worsening bronchospasm/airflow obstruction unresponsive to standard therapy Incidence • Most common chronic illness in childhood Etiology • Triggers • Extrinsic: Allergic/immunologic factors • Intrinsic: Infectious • Exercise induced

www.uhn.ca

• Severity Classification • Intermittent, mild persistent, moderate persistent, severe persistent • See National Heart, Lung, Blood Institute Guidelines for more information and the ʻGuidelines for the Diagnosis and Management of Asthmaʼ at: http://www.nhlbi.nih.gov/guidelines/asthma/

• Symptoms • Cough, especially at night • Tachypnea • Shortness of breath • Wheezing, forced and prolonged expiratory phase • Accessory muscle use • Tachycardia • Hypoxia • Pulsus paradoxus (moderate/severe exacerbations) • *Fever, if associated with infectious trigger • Diagnosis • History and physical exam • Chest radiograph: Hyperinflation, flattened diaphragms, peribronchial thickening, narrowed cardiac silhouette • Additional studies (e.g. arterial blood gas, lactate, electrolytes may be helpful in moderate/severe cases)

www.pedsccm.wustl.edu

• When following arterial blood gases or other carbon dioxide measurements, anticipate hypocarbia when compensating well for exacerbation • A normal or rising carbon dioxide measurement is worrisome

Hyperinflation, narrowed cardiac silhouette

Management

• Inhaled Beta2 agonists (albuterol, levalbuterol) • Bronchial smooth muscle relaxation • Reduce antigen induced histamine release • Increase mucociliary transport • Intermittent dosing (MDI or nebulized); typically every 20 minutes for one hour. Continuous for refractory exacerbation • Corticosteroids • Decreases inflammation associated with chronic and acute airway inflammation • May be given intravenously or enterally • 2‐4 hours to take effect • Therapy > 5‐7 days requires taper

Management – Continued •Anticholinergics (e.g. ipratropium bromide) • Promotes bronchodilation • Used most frequently in the Emergency Department to prevent hospitalization •Admission criteria • Symptoms after observation for 60 minutes • Oxygen requirement • Short acting beta agonists > every 2‐3 hours • Prior intensive care unit admissions

Adjunctive Therapies • Magnesium sulfate • Physiologic calcium antagonist; causes smooth muscle relaxation • May be administered continuously or intermittently, IV • Most common adverse reaction is hypotension • Intravenous beta agonist (e.g Terbutaline) • Bolus, +/‐ continuous infusion • ECG monitoring • Methylxanthines (e.g aminophylline, theophylline) • Promotes smooth muscle relaxation through unknown mechanism • Narrow therapeutic index; requires serum drug level monitoring • High side effect profile (nausea, vomiting, seizures, abdominal discomfort)

Adjunctive Therapies ‐ Continued

•Non‐Invasive ventilation •Heliox • Low density gas • Promotes laminar flow • Facilitates delivery of oxygen and aerosolized medications Turbulent flow Laminar flow

•Invasive ventilation • Use with caution Risk for air leak syndromes • Allow permissive hypercapnia • May require muscle relaxation

©202 0 PEARL: Ketamine typically used for induction in asthmatics requiring intubation due to its bronchodilatory effects.

Background •Commonly known as ʻwhooping coughʼ •Making a comeback; immunity does not appear to be permanent Etiology •Bordetella pertussis; Aerobic gram negative coccobacillus •Primarily toxin mediated disease • Toxin paralyzes cilia, causes inflammation in the respiratory tract •Transmitted by aerosolized droplets; highly contagious •It remains a significant health threat, in particular to infants and young children, who are at greatest risk for disease complications •Older children and adults are often the reservoir

• Presentation/Stages • Catarrhal stage: • Nasal congestion • Rhinorrhea • Low grade fever • Non‐productive cough • Paroxysmal stage: • Cough more severe at night • Cough changes into spasms of paroxysmal coughing • Stridor or “whooping” • Gasping, gagging with feedings • *Young infants often do not present with ʻwhoopingʼ • Young infants often present w/ apnea • Paroxysmal stage may last 1‐2 weeks • Convalescence Stage: Cough may last up to 6 ‐ 10 weeks

• Complications • Pneumonia • Atelectasis • Otitis media • Encephalopathy • Seizures • Death • Highest mortality rate is in infants < 6 months of age

• Diagnosis • Characteristic clinical history • Nasopharyngeal secretions • Direct fluorescence antibody (DFA) • Polymerase chain reaction (PCR) • Culture • CBC • Leukocytosis • Lymphocyte predominance; 50 ‐80,000/cm3 • Chest radiograph • May be unremarkable or demonstrate findings of small airway disease

• Management • Droplet isolation • Antibiotics • Start prior to diagnostic confirmation • Recommendations varying based on age. See www.cdc.gov/pertussis for latest recommendations • Erythromycin, clarithromycin, and azithromycin all acceptable • Avoid erythromycin in infants < 1 month of age (risk for hypertrophic pyloric stenosis) • Supportive care • Hospitalization if evidence of pneumonia or risk for apnea • Respiratory and oxygen saturation monitoring • Oxygen if necessary • Humidification/suctioning • Mechanical ventilation if significant apnea or alveolar disease

•Post‐exposure Antimicrobial Prophylaxis • Provided to all household contacts of a pertussis case • Provided to persons within 21 days of exposure to a patient infected with pertussis who are at high risk of severe illness or have close contact with a person at high risk for severe disease (e.g. infants, women in 3rd trimester of pregnancy, immunocompromised persons)

http://www.cdc.gov/pertussis/outbreaks/PEP.html

www.cdc.gov/pertussis

• Definition • Acute severe inflammation of epiglottis • Results in displacing epiglottis posteriorly • May obstruct breathing • Airway emergency! • Etiology • Haemophilus influenzae • Has decreased steadily since Hib vaccine • May be caused by other bacteria or viruses; Staphylococcus aureus, Streptococcus pneumoniae, Group A Streptococcus

www.sickkids.on.ca

• Epidemiology • Most commonly occurs in children 1‐5 years of age • Clinical Manifestations • Sudden onset of symptoms • Sore throat • High fever • Respiratory distress • Difficulty swallowing/drooling/dysphagia • Muffled voice/ʼhot potatoʼ voice • Tripod position • Anxious appearing child • Stridor

www.nlm.nih.gov

• Diagnostic Evaluation • Lateral neck radiograph: Enlarged epiglottis and distended hypopharynx (ʻthumbprint signʼ) • Direct laryngoscopy: Beefy red, swollen epiglottis • Blood and/or throat culture may reveal offending pathogen; obtained after airway is secure or no longer critical • Complete blood count may reveal leukocytosis (non‐specific)

www.learningradiology.com

• Management • Noxious stimuli must be avoided • Let child assume position of comfort • Consult otolaryngology or anesthesia for possible intubation • If not intubated, provide humidified oxygen • Antibiotics • Third generation cephalosporin or third generation cephalosporin plus vancomycin if penicillin‐resistant pneumococci or methicillin resistant Staphylococcus aureus is suspected • 7 ‐10 days of therapy • Consider systemic steroids • Intravenous fluids to prevent dehydration

• Definition • Abscess in the retropharyngeal space following trauma or primary infection

• Epidemiology/Etiology • Most common in children 1 – 5 years of age • Infections are typically polymicrobial • Common pathogens include: Streptococcus pyogenes, Staphylococcus aureus, and Haemophilus species

www.neuroradiologyportal.com Retropharyngeal Abscess

• Presentation • History may include recent intubation, oral foreign object, dental procedure, infection of structure draining into retropharyngeal space • Sore throat • Fever • Dysphagia • Trismus • Neck swelling • Muffled voice; ʻhot potatoʼ voice • Also, cervical adenopathy, retropharyngeal bulge, stridor, torticollis, drooling

• Diagnostic Evaluation • History and physical exam • Complete blood count and differential; leukocytosis • Blood cultures rarely positive • Lateral neck film: Widening of retropharyngeal soft tissues; widening of preverterbal space. May help differentiate RPA from epiglottitis • CT scan of neck with IV Contrast: Identifies extent of infection and differentiate abscess from cellulitis • Chest radiograph: Evaluates for mediastinal expansion

Raju, R. & Pigoy, G.P. (2009). Deep Space Neck Infection in the Pediatric Population. In K. D. Pereira & R. B. Mitchell. (Eds). Pediatric Otolaryngology for the Clinician doi. 10.1007/978-1-60327-127-1_28©. Humana Press.

• Management • Avoid noxious stimuli • Supplemental oxygen • Assure secured airway • May require intubation in severe cases • Surgical drainage required for airway compromise, abscess > 2cm, failure to improve on IV antibiotics within 24 hours • Antibiotics • Broad spectrum • Coverage for aerobic and anaerobic organisms • If patient afebrile and improving, can change to enteral antibiotic to complete 14 day course

• Definition • A local cellulitis that progresses first to phlegmon, then abscess; suppurative adenitis is most common • Etiology/Epidemiology • Most common deep neck infection in children • Occurs most commonly in older children/adolescents • Infections are most commonly polymicrobial • Common pathogens include: Streptococcus pyogenes, Staphylococcus aureus, and Haemophilus species

• Clinical Manifestations • Sore throat; pain often radiates to ear • Tender glands of throat and/or jaw • Fever • Chills • Facial swelling • Difficulty/discomfort with opening mouth; may refuse to eat or drink • Drooling • Halitosis • Voice may be muffled or difficult to understand; ʻhot potatoʼvoice • Swollen tonsils with uvula deviation • Swollen tissues may obstruct the airway, which can lead to a life threatening emergency

www.myhealth.gov

• Diagnostic Evaluation • Exam of throat with swelling of tonsil(s), palate, throat • Often, displacement of uvula to opposite side • Complete blood count and differential; leukocytosis • Blood cultures rarely positive • Throat culture‐evaluate for group A Streptococcus • Neck abscess incision and drainage; send for culture • CT scan with IV contrast; evaluate for extent of infection and differentiate from cellulitis

www.medicine.ucsd.edu

• Management • Avoid noxious stimuli • Surgical drainage; consider if abscess is > 2cm or if failure to respond to antibiotic therapy. Required for airway compromise • Intravenous antibiotics with ampicillin‐sulbactam or clindamycin • Consider coverage for resistant organism (e.g. vancomycin) • Analgesics • Assess hydration status; may require IV hydration

Lower respiratory tract invasion by pathogen resulting in respiratory symptoms, hypoxia. May be due to inhalation, aspiration, epithelium invasion, hematogenous spread

• Symptoms: Cough, fever, tachypnea, difficulty breathing. May also be associated with abdominal pain

Etiology: Can often be determined by child’s age and contributing factors • Neonates: Most often bacterial; associated with pathogens in birth canal – Group B streptococcus, Klebsiella, Escherichia coli, Listeria monocytogenes • Last onset neonatal: Staphylococcus, Streptococcus • Older infants/toddlers (30 days – 2 years): Viral etiology • 2‐5 years of age – virus still common; increase in Streptococcus pneumoniae and Haemophilus influenzae type B • 5‐13 years of age – Mycoplasma pneumonia; Streptococcus pneumoniae most common identified pathogen

Evaluation: • History: preceding symptoms, travel history, sick contacts, immunization status, overall health of child, choking episode • Rapid testing – non‐invasive (e.g. viral panel; can minimize unnecessary antibiotics) • Severe disease/toxic appearance – CBC, electrolytes, blood culture • Inflammatory markers do not help differentiate viral vs bacterial etiology in children; can be used for trends in children with severe illness • Chest radiograph: No clear guidelines on which cases it is indicated; determined by age/severity of illness

Treatment • Targeted for expected pathogen based on child’s age and severity of illness – If antibiotics are indicated: Neonates: Ampicillin, plus aminoglycoside or third generation cephalosporin; infants > 3 months and young children: high dose amoxicillin or beta lactam; school‐age: macrolide • Respiratory supportive measures as indicated by examiniation • Antipyretics • Fluid management, as needed

Parapneumonic effusion • Common in bacterial pneumonia (generally small); will resolve with appropriate antibiotic therapy. May require drainage if moderate/large in size. Commonly transudative. Pleura is inflammed; leakage of proteins. Fluid is sterile with low leukocyte count Empyema • Spread of infection (bacteria) into the pleural fluid • Fluid is purulent when drained • Antibiotics mainstay of therapy. May require thoracotomy/chest tube +/‐ fibrinolytic administration, video‐assisted thoracentesis (VATS)

• Definition • Complete cessation of airflow through nose and mouth despite continued inspiratory and expiratory efforts • Absence of airflow may be brief • With severe forms, may be prolonged and associated with oxygen desaturation, cardiac deceleration, cardiac arrhythmia and carbon dioxide retention

• Etiology • Most commonly, hypertrophy of tonsils +/‐ adenoids • Children with craniofacial abnormalities are at higher risk • Micrognathia • Maxillary hypoplasia • High arched palate • Other midface defects • Children with neurologic abnormalities are at higher risk • Abnormalities with increased or decreased muscle tone • Other genetic associations with higher risk • Trisomy 21 • Achondroplasia • Prader‐Willi

www.path.upmc.edu

• Clinical Manifestations • Nighttime symptoms • Snoring, characteristic feature • Often associated with pauses and snorts • Difficulty breathing during sleep • Restless sleep • Morning headaches • Excessive thirst upon awakening • Nightmares • Sleep terrors • Frequent nocturnal waking • Sleep‐related enuresis

www.health.yahoo.com

• Daytime symptoms • Excessive daytime sleepiness • Hyperactivity • Attention span difficulties • Poor school performance • Behavioral abnormalities • Frequent upper respiratory tract infections • Other • Failure to thrive • Obesity • Severe cases • Pulmonary hypertension • Cor pulmonale

• Diagnosis • Clinical symptoms • Enlarged tonsils on exam • Sleep study • Consider ECHO and 12 lead ECG • Treatment • Tonsillectomy and adenoidectomy; in those children with hypertrophy • Does NOT cure all children; especially obese children • Other identifiable causes of upper airway obstruction should be identified and appropriately managed • Nasal or oronasal continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP) • Tracheotomy • Curative, but reserved for most severe patients

www.gent.med.kyushare‐u.ac.jp

Background •Most common congenital anomaly of trachea •More common in premature infants •Associated with feeding problems Definition •Upper airway (trachea) lacking cartilaginous rigidity (e.g. ʻfloppyʼ)

• Clinical presentation • Symptoms may appear in first week of life, though are commonly delayed 2 – 3 months • Expiratory stridor and cough are most common symptoms • Symptoms worsen with agitation, crying, feeding, or upper airway infections • Harsh, barky cough

• Diagnostic evaluation • History and physical exam • Flexible bronchoscopy; performed during spontaneous respiration • CT scan; provides information on aortic arch anatomy, airway compression, evaluates for vascular rings • Fluoroscopy; evaluates underlying anatomy • Laryngoscopy; evaluated for laryngomalacia

• Management • Observation (most cases) • Symptom resolution at or before 18 months of age in most children • Tracheostomy and continuous positive airway pressure in severe cases • Aortopexy for severe cases; suspends the anterior trachea reducing airway compression

• Background • Result of injury to the alveolar capillary membrance

• Etiology • Preceded by a variety of diseases • Direct lung injury • Shock • Sepsis • Drowning • Trauma • Aspiration • Pulmonary contusion • Transfusion‐related lung injury (TRALI) • Cardiopulmonary arrest

• Stages • Exudative • Injury to the alveolar capillary barrier • Increased pulmonary congestion • Pulmonary edema • Depletion of endogenous surfactant • Proliferative • Proliferation of type II pneumocytes, fibroblasts, and myofibroblasts • Healing begins • Heterogenous lungs‐ interspersed with normal lung function • Fibrotic • Lungs begin to remodel with collagenous tissue • Fibrotic scarring • Complications of treatment begin to surface • Progressing through these stages leads to pulmonary hypertension, bronchoconstriction, hypovolemia, and increased pulmonary vascular resistance

• Clinical Manifestations • Tachypnea • Tachycardia • Respiratory distress • Hypocapnia (initially) • Decreased aeration • Rales, wheezing • Diagnostic Evaluation

• Arterial blood gas: PaO2/FiO2 ratio of < 200 (equation used to predict ʻshuntʼ) • Chest radiograph: Bilateral infiltrates • ECHO: Evaluate for cardiogenic etiology of pulmonary disease

www.emedicine.com

• Management • Supportive care • Respiratory support; oxygen, mechanical ventilation • Cardiovascular support, as needed (i.e. inotropes) • Nutritional/fluid support • Often require diuretic therapy • Consider corticosteroid administration • Consider surfactant • Consider nitric oxide; results in capillary and pulmonary vasodilation • Complications • Ventilator associated lung injury • Permissive hypercapnia, low tidal volume, avoid PIP > 30 • Oxygen toxicity

www.emedicine.com

• Definition • Inherited chronic multi‐system disorder caused by mutations in the cystic fibrosis transmembrane regulator CFTR gene; over 1500 gene mutations identified • Etiology • Autosomal recessive genetic disease • Incidence • 1:3300 live births in Caucasians in US • Less frequent in other populations

• Diagnosis • Prenatal • Amniocentesis • Chorionic villus sampling • Echogenic bowel may be noted on ultrasound • Neonatal Screen • Performed in most US states; does not test for all gene types • Postnatal • Clinical presentation • Sweat chloride testing, initial test • Confirmation testing • Second sweat chloride or genotyping • Some centers perform nasal potential difference • Gene testing

www.discovery‐diagnostics.com

• Pathophysiology • Defective gene makes an abnormal protein • Impairs movement of salt and water across the epithelial cell wall in the exocrine glands • Leads to sticky secretions • Affection body systems include: Lungs, pancreas, intestine, liver, sinuses, reproductive tract, sweat glands

Presentation • Newborn screen • General; poor growth • Meconium ileus • A neonatal bowel obstruction (15‐20% of affected) • Prolonged cholestatic jaundice • Frequent lower respiratory tract infections

• Clinical Manifestations • Progressive pulmonary disease • Pulmonary exacerbations • Increased cough • Sputum production • Hemoptysis • Weight loss • Decreased pulmonary function • Allergic bronchopulmonary aspergillosis • Nasal polyposis • Sinusitis • Poor growth/development • Gastroesophageal reflux • Fat soluble vitamin deficiency (A,D,E,K) • Distal intestinal obstruction • Due to dehydrated, bulky feces • Chronic constipation • Elevated liver function tests (gallstones, cirrhosis) • Infertility • Men (absence of vas deferens)

www.pncl.co.uk

• Management • Respiratory • Airway clearance techniques • Airway hydrators (e.g dornase alpha, hypertonic saline) • Nutritional therapy • High calorie diet • Unrestricted fat • Pancreatic enzymes • May need supplemental G tube feeds • Endocrine • Frequent blood glucose monitoring; may require insulin • Hepatic • Improves with ursodeoxycholic acid • Some require liver transplantation

www.wellesley.com

• Gastrointestinal • Stool softeners • Infection • Frequent monitoring of respiratory tract • Pulmonary exacerbations • Antibiotics • Increased frequency of chest physiotherapy • Lung transplantation • Option for some • Prognosis • 90% of deaths secondary to respiratory failure • Median life expectancy continues to increase (~ 37 years)

• Definition • Materials traveling in the blood stream become lodged in the pulmonary arterial bed • Etiology • Thromboembolism, most common • Incidence • Rare in infants and children • Under‐diagnosed?

www.benlovejoy.com

• Risk factors • Trauma • Post‐surgical • Presence of central venous catheter • Immobility • Heart disease • Infection • Dehydration • Collagen vascular disease • Shock • Obesity • Ventriculo‐atrial shunt • Oral contraceptive use

• Pathophysiology • Decreased perfusion to affected alveolar units distal to emboli • Increased alveolar dead space • Impaired ability to eliminate carbon dioxide • Ventilation‐perfusion mismatch

www.emedicine.com

• Clinical Manifestations • VARIED • Pleuritic chest pain • Dyspnea • Patient experiencing sense of doom • Cough • Rales • Tachycardia • Fever • Diaphoresis • Phlebitis • Wheezing • Hemoptysis • Severe cases, hemodynamic instability

• Diagnosis • Arterial blood gas • Respiratory alkalosis, arterial hypoxemia, or normal • Elevated D‐Dimers • Chest X‐Ray; may be normal • Ventilation‐Perfusion Scan (VQ Scan) • Displays regional blood flow and ventilation • Non‐invasive • Normal scan does not completely exclude possibility of PE • Reports are interpreted in terms of probability • Helical spiral CT Scan • Particularly useful in patients with concurrent lung disease • Results somewhat dependent on experience of radiologist • Pulmonary angiography • Invasive and expensive, but relatively safe

www.gpnotebook.co.uk

• Additional Considerations • If familial or acquired coagulation disorders are suspected • Fibrinogen, protein C, protein S, antithrombin III, factor V leiden, lupus anticoagulant, anticardiolipin antibody • Evaluate for deep vein thrombosis • Femoral and inferior vena cava are most common sites • Doppler ultrasounds are useful non‐invasive tests • Venogram, if high suspicion and negative ultrasound

• Management • ABCs • Vasopressor support, if indicated • Anticoagulation (heparin, low molecular weight heparin, coumadin) • Thrombolysis (urokinase, streptokinase, recombinant tissue plasminogen activator) • Thrombectomy, if persistent hemodynamic compromise • Inferior vena cava filters, indicated for patients with recurrent PE despite medical therapy

• Definition • Congenital or acquired communication between the trachea and esophagus • Several types based on location of fistula • Five types defined • Etiology • Congenital malformation • Acquired forms • Blunt chest or neck trauma, excessive pressure in tube cuff pressure in intubated patients

www.pennhealth.com ©202 0 Tracheoesophageal Fistula

• Additional Considerations • Associated Genitourinary defects • Renal agenesis • Horseshoe kidney • Polycystic kidney disease • Ureteral/urethral malformations • Hypospadias • Associated Gastrointestinal defects • Imperforate anus • Duodenal atresia • Malrotation • Intestinal malformation • Meckelʼs diverticulum

www.brown.bms.edu ©202 0 Tracheoesophageal Fistula

• Additional Considerations • Associated Musculoskeletal defects • Hemivertibrae • Radial anomalies • Poly or Syndactyly • Rib malformations • Scoliosis • Lower limb defects • *May be associated with VATER syndrome • Vertebral/Vascular • Anal atresia • Tracheoesophageal fistula • Esophageal atresia • Renal anomalies/radial anomalies

• Clinical Manifestations • Coughing/choking • Symptoms worsen with feeding • Abdominal distention; due to secondary air collection in stomach • Diagnosis • Prenatal • Ultrasound (polyhydramnios, absence of fluid filled stomach, distended esophageal pouch) • Post‐Natal • Conventional radiographs; air distended esophageal pouch, tracheal compression, NG tube curled in pouch • Contrast studies not necessary unless H‐Type fistula is suspected (TEF with no associated atresia) • Acquired TEF • Instillation of contrast media into esophagus or during direct visualization by flexible endoscopy or bronchoscopy

• Management • Pre‐operative • Reduce risk of aspiration • Continuous suctioning of blind esophageal pouch • Head of bed elevated • Nutritional/fluid support • Intubation/ventilation, as needed • Consider antibiotics, if lower respiratory tract infection is suspected • Gastric decompression • Gastric acid suppression

• Operative Repair • First few days of life; primary repair in otherwise healthy infants • Delayed in infants with low birth weight, pneumonia, or other anomalies

• Blood Gas Analysis • Utility • Measurement of oxygenation • Measurement of ventilation • Measurement of acid/base status • Indications • Symptoms of oxygenation, ventilation or acid/base imbalance • Used to monitor patients requiring respiratory support measures

• Normal Values • pH 7.35 – 7.45 • CO2 35‐45 • Bicarbonate 22‐26 • PaO2 80‐100 • *Take into account patient status, temperature, blood gas sample, amount of oxygen being administered

•Steps 1. Look at each number individually and label it (ex: high pH = alkalemia) 2. Evaluate oxygenation – PaO2 & SaO2 3. Determine acid‐base status 4. Determine if acid base status is metabolic or respiratory (may reflect one imbalance or may be mixed) 5. Determine if a compensatory response has occurred

• pH 7.52/PCO2 28/ PaO2 94/Bicarbonate 24

• Label each, evaluate oxygenation, determine acid‐base status, respiratory or metabolic, any compensation? • What conditions may be associated with this blood gas?

• pH 7.31 /PaCO2 62/ PaO2 81 /Bicarbonate 23 • Label each, evaluate oxygenation, determine acid‐base status, respiratory or metabolic, any compensation? • What conditions may be associated with this blood gas?

Respiratory Acidosis: Retention of CO2 • Hypoventilation • CNS depression (anesthesia, narcotics, sedatives, drug overdose) • Respiratory neuromuscular disorders • Trauma: spine, brain, chest wall • Restrictive lung diseases • Chronic obstructive pulmonary disease • Acute airway obstruction (late phases)

Respiratory Alkalosis: Hyperventilation •Hypoxemia • Anxiety, fear •Pain •Fever •Stimulants • CNS irritation (e.g., central hyperventilation) • Excessive ventilatory support (bag‐valve‐mask, mechanical ventilation)

Increased Acids • Diabetic ketoacidosis • Renal failure • Lactic acidosis • Drug overdose (salicylates, methanol, ethylene glycol) Loss of Base • Diarrhea • Pancreatic or small bowel fluid loss

Gain of Base • Excess ingestion of antacids • Excess administration of sodium bicarbonate • Citrate in blood transfusions Loss of Metabolic Acids • Vomiting • Nasogastric suctioning • Low potassium and/or chloride • Diuretics (loss of chloride and/or potassium)

• Goal of therapy • Ensure adequate oxygenation and minute ventilation • Minute ventilation = Respiratory rate X tidal volume

• Non‐invasive ventilation • Continuous positive airway pressure (CPAP) • Bilevel positive airway pressure (BiPAP®) • Prongs • Mask • Nasal pillows • High Flow oxygen devices • Invasive Ventilation

• Provides positive pressure ventilation without the use of endotracheal tube • Increases surface area available to participate in gas exchange • Can ʻstentʼ open upper airways

• Benefits • No risk of airway trauma • Less risk for aspiration pneumonititis • Decreased risk for hospital acquired pneumonia • Decreases work load on respiratory muscles, but not completely • Requires less sedation and analgesia • Less expensive

www.hhonline.com

• Caveats • May be difficult for infants to ʻtriggerʼ machine • Appropriate size mask availability • Too large of leak • Not for patients with apnea • Can cause significant agitation in some children

• High Flow Nasal Cannula

High Flow Nasal Cannula • Has gained significant popularity over last decade • Delivers oxygen flow with high levels of humidity • Reduces dryness • Allows flows of up to 60 liters per minute (adult‐size patients) • Oxygen delivery is titratable • Exact delivery of ‘pressure’ with this mode is unknown

• Continuous Positive Airway Pressure (CPAP) • Provides one set pressure being delivered to the patient throughout the respiratory cycle • This pressure is constant throughout the respiratory cycle

www.kenbmiller.com Non‐Invasive Ventilation

• Bilevel Positive Airway Pressure (BiPAP) • Provides two levels of pressure • One level of pressure (higher) during inspiration • One level of pressure (lower) in between respirations • Keeps lung open during the entire respiratory cycle • Can be set in spontaneous or time cycled mode

www.our‐sma‐angels.com

• Used when patients cannot be adequately managed with non‐invasive modalities • Requires placement of endotracheal tube • Benefits • Ability to provide positive pressure and oxygen • Ability to override patientsʼ work of breathing • Risks • Difficult airway (upon intubation) • Tracheal/vocal cord injury • Subglottic stenosis • Muscle weakness/atrophy • Pneumothorax/pneumomediastinum

www.xmlportfolio.com

• After intubation, check for proper placement! • Chest rise • Breath sounds • Presence of carbon dioxide by capnogram/capnography • Condensation in ETT • Chest radiograph

• Proper placement

• Capnography • Blood gases – ABG, VBG, CBG • Transcutaneous CO2 • Exhaled tidal volumes • Peak pressures • AutoPEEP • Flow volume loops

• Normal

• What happens to the waveform when there is a prolonged exhalation phase/air trapping (e.g. asthma)? • What is going on when the waveform appears normal one second and disappears the next?

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• Commonly used modes… • Several others are available

• Many modes available • Common modes • Pressure support only • No rate • Two levels of pressure (one on inspiration and one at end of expiration and in between breaths) • Pressure control pressure support • Set pressure with each breath • Amount of volume (size of breath) depends on compliance of lungs • Volume control pressure support • Set volume delivered with each breath • Amount of pressure required to deliver the pressure provides information on lung compliance • High pressures to achieve set volume indicate poor lung compliance

• High frequency oscillatory ventilation – HFOV/Oscillator – Oscillates around higher mean airway pressure (MAP) – Piston‐driven; very small tidal volume – Useful in severe hypoxia requiring high MAP • Jet ventilation – Always run in tandem with conventional ventilator to generate PEEP and sigh breaths – Exhalation is passive – Useful in airleak syndromes • Others

©202 0 Settings Adjusted to Eliminate More Carbon Dioxide • Ventilator rate • In pressure control mode • Pressure control • Adjusts size of vent breath delivered • In volume control mode • Tidal volume • Adjusts size of vent breath delivered • In any mode • Inspiratory time • Shorter time for inspiration allows more time for exhalation

• Increase FiO2 • Increase Positive end expiratory pressure (PEEP) • Increase inspiratory time • Increases amount of time ʻunder the curveʼ; increases mean airway pressure • Sometimes, pressure control/tidal volume increase (also increases area/time under the curve)

Why not just use high amounts of inspired oxygen in hypoxia?

• In times of acute decompensation: • Always disconnect patient from ventilator • Hand ventilate with 100% oxygen • Is it a problem with patient? • Mucus plug/secretions? • Displaced endotracheal tube? • Aspiration event? • Pneumothorax? • Deterioration in status? • Is it a mechanical problem? • Ventilator malfunction?

• Artificial airway • Plastic or metal • Surgically placed just below the larynx in the trachea • Bypasses mouth and upper airway • Reversible

• Failure of mask ventilation • Maintain patent airway • Bypass airway obstruction • Tumor, foreign body, edema • Facilitate secretion removal • Severe tracheo or bronchomalacia • Muscle weakness

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• Monitor • Respiratory status • Vital signs • Color, consistency of secretions • Fresh trach tube (< one week old) • Maintain tract • Stay sutures • Humidification • Loosens trach secretions

• Infection • Tracheoesophageal fistula • Tracheal stenosis • Erosion into inominate artery

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A 2‐year‐old female presents with a 2‐day history of fever, cough, tachypnea. She has had decreased oral intake for the last 24 hours. No vomiting, diarrhea, rhinorrhea, congestion. She lives with her parents and 4‐year‐old brother. She attends daycare 4 days per week. She has eczema and no other past medical history. No prior surgeries or hospitalizations.

Her initial vital signs are: Temp: 39.0 C Heart Rate: 119 beats per minute Respiratory Rate: 38 breaths per minute Blood Pressure: 89/42 Oxygen Saturation: 91% room air

What are the top differential diagnoses for this toddler?

What evaluation are you considering?

• Does your differential diagnosis change after obtaining this radiograph?

• What is included on your treatment plan?

• She is started on high flow nasal cannula 50% 8liters/minute flow. Over the next 2 days she has continued with fever, tachypnea, and intermittent fevers.

• Would you expect that she is demonstrating clinical improvement at this point in her course?

• Would you obtain any other studies?

• Part of your continued care includes obtaining an additional chest radiograph.

• How does this impact your treatment plan? • What are the next steps in diagnostic evaluation and treatment plan?

• Four days after further intervention and continued care, she is demonstrating consistent improvement • Ten days after admission, she is discharged home on room air, eating well and playful • Would you anticipate that her chest radiograph is normal at the time of discharge? • Do you anticipate long term consequences of her illness and hospitalization?

A 2‐year‐old is post‐operative day #0 from placement of a tracheostomy. The child experiences an acute desaturation episode and is the chest is not moving despite ventilation with hand bagging. What is the most appropriate intervention? A. Remove trach, pull stay sutures toward feet, reinsert tracheostomy B. Remove trach, place gauze over trach site, and bag over mouth/nose C. Readjust trach without removing, keep bagging, call for help D. Readjust trach without removing, keep bagging, and prepare for intubation from above

B. Remove trach, place gauze over trach site, and bag over mouth/nose

While managing a 10‐month‐old intubated from pneumonia, you note the peak pressures on ventilator have increased 20% since the last time you examined the patient. Which of the following is likely responsible for the change in pressure? A. Decreased lung compliance B. Increased lung compliance C. Decreased bronchospasm D. Increased ventilator dead space

A. Decreased lung compliance

A 10 year‐old child is admitted with diabetes ketoacidosis (DKA). What would you expect that her respiratory rate be?

A. 6 breaths per minute B. 10 breaths per minute C . 18 breaths per minute D. 40 breaths per minute

A 10 year‐old child is admitted with diabetes ketoacidosis (DKA). What would you expect that her respiratory rate be?

D. 40 breaths per minute

A 1‐month‐old male is intubated in the ICU. You are called to evaluate and intervene on the following blood gas: pH 7.31/PaCO2 64/PaO2 91/Bicarbonate 24. Which of the following is the most appropriate ventilator adjustment?

A. Increase ventilator rate B. Increase the FiO2 C. Increase the positive end expiratory pressure (PEEP) D. Increase the inspiratory time

A. Increase ventilator rate

4‐year‐old child was diagnosed with Streptococcal pneumoniae pneumonia 4 days ago and was stated on high dose amoxicillin. Today, he develops new increased respiratory distress and fever. What do you anticipate may be going on? a. Aspiration secondary to frequent coughing b. Parapneumonic effusion/empyema c. Secondary sinusitis d. New viral infection

B. Parapneumonic effusion/empyema

A 10‐year‐old male is intubated in the ICU. You are called to evaluate and intervene on the following blood gas: pH 7.41/PaCO2 38/PaO2 61/Bicarbonate 24. Which of the following is the most appropriate ventilator adjustment? A. Increase ventilator rate B. Increase the pressure control C. Increase the positive end expiratory pressure (PEEP) D. Increase the expiratory time

C. Increase the positive end expiratory pressure (PEEP)

Which of the following findings is expected on the chest radiograph of a child with status asthmaticus? a. Pleural effusion b. Widened cardiac silhouette c. Flattened diaphragms d. Hypoexpansion

Which of the following findings is expected on the chest radiograph of a child with status asthmaticus?

C. Flattened diaphragms

Which of the following are the most likely pathogens in an 8 month old infant with 3 days of upper respiratory tract infection symptoms and low grade fever, now presenting with harsh cough and stridor worse at nighttime? a. Respiratory syncytial virus and adenovirus b. Human metapneumovirus and influenza c. Parainfluenza type 1 & 2 D Rhinovirus and parainfluenza type 3

C. Parainfluenza type 1 & 2

Which of the following may be a side effect of magnesium sulfate infusion as adjunct therapy in a child with status asthmaticus?

A. Muscle fasciculation B. Hypotension C. Torsades de Pointe D. Hyperglycemia

Which of the following may be a side effect of magnesium sulfate infusion as adjunct therapy in a child with status asthmaticus?

B. Hypotension

An adolescent intubated for asthma has persistent hypercarbia on current ventilator settings. Which of the following strategies may be used to enhance removal of C02?

A. Decrease PEEP B. Increase inspiratory time C. Decrease ventilator rate D. Increase Fi02

An adolescent intubated for asthma has persistent hypercarbia on current ventilator settings. Which of the following strategies may be used to enhance removal of C02?

C. Decrease ventilator rate

4‐year‐old child diagnosed with viral pneumonia 4 days ago, develops a new fever and worsening respiratory symptoms. What do you suspect may be going on? a. Aspiration secondary to frequent coughing b. Development of meningitis c. Undiagnosed cystic fibrosis d. Bacterial superinfection

4‐year‐old child diagnosed with viral pneumonia 4 days ago, develops a new fever and worsening respiratory symptoms. What do you suspect may be going on?

D. Bacterial superinfection

©202 0 Question A 2 year old has had cold symptoms for the past week and now has significant stridor which increases with mild activities. He is evaluated in the ED and found to be hypoxic. What would be an expected finding on the neck radiograph of this child? a. Thumbprint Sign b. Increased hypophayngeal space c. Brudzinskiʼs Sign d. Steeple Sign

©202 0 Answer: A 2 year old has had cold symptoms for the past week and now has significant stridor which increases with mild activities. He is evaluated in the ED and found to be hypoxic. What would be an expected finding on the neck radiograph of this child?

d. Steeple Sign

Which of the following best describes the therapy for an infant with low grade fever, rhinorrhea, cough, tachypnea, wheezing, positive for respiratory syncytial virus? a. Inhaled bronchodilators always indicated b. Antibiotics are foundation of management c. Corticosteroids initiated immediately d. Oxygen, suctioning, supportive care

Which of the following best describes the therapy for an infant with low grade fever, rhinorrhea, cough, tachypnea, wheezing, positive for respiratory syncytial virus?

D. Oxygen, suctioning, supportive care

Which of the following management strategies is recommended when managing an infant with pertussis? a. Steroids to reduce the duration of cough b. Chemoprophylaxis is recommended for all household contacts c. Erythromycin is indicated only in infants < 1 month of age d. Antibiotics are not recommended

Which of the following management strategies is recommended when managing an infant with pertussis? b. Chemoprophylaxis is recommended for all household contacts

©202 0 Question Which of the following organisms are common pathogens in epiglottis since the inception of the Haemophilus Influenza B vaccine? a. Staphylococcus aureus and Streptococcus pneumoniae b. Pseudomonas aeruginosa and Klebsiella pneumonia c. Escherichia coli and Streptococcus pyogenes d. Streptococcus viridans and Respiratory syncytial virus

Which of the following organisms are common pathogens in epiglottis since the inception of the Haemophilus Influenza B vaccine? a. Staphylococcus aureus and Streptococcus pneumoniae

• A 10 year‐old child is admitted with diabetes ketoacidosis (DKA). Her mother is worried about her deep, tachypneic respirations. What is an appropriate response to her mother about her respiratory rate?

• A. She will need to be intubated to slow her respiratory rate • B. This is a normal response to her acidosis and will resolve as her condition improves • C. This is not associated with her DKA and there must be a concomitant respiratory process going on • D. Steroids will be administered to reduce ongoing inflammation

A 10 year‐old child is admitted with diabetes ketoacidosis (DKA). Her mother is worried about her deep, tachypneic respirations. What is an appropriate response to her mother about her respiratory rate?

B. This is a normal response to her acidosis and will resolve as her condition improves

When evaluating a 19‐month‐old male with acute onset of high fever, respiratory distress, drooling, and stridor, which of the following is an expected finding on a lateral neck film?

a. Thumbprint sign b. Steeple sign c. Kerley‐B lines d. Narrowed hypopharyngeal space

©202 0 Answer: When evaluating a 19‐month‐old male with acute onset of high fever, respiratory distress, drooling, and stridor, which of the following is an expected finding on a lateral neck film? a. Thumbprint sign

©202 0 Question A 13 month old, weighing 11 kg, presents with stridor and low grade fever. The child had been ill for 5 days with cold symptoms and cough. Parents attempted to manage the child with humidified air and acetaminophen, but his work of breathing increased. The child’s oxygen saturation on room air is 94%. What is the recommended first step in treatment for this child? a. Dexamethasone 22 mg given IV b. Dexamethasone 6.6mg given IM c. Nebulized albuterol inhalation d. Nebulized 3% saline inhalation

©202 0 Answer: A 13 month old, weighing 11 kg, presents with stridor and low grade fever. The child had been ill for 5 days with cold symptoms and cough. Parents attempted to manage the child with humidified air and acetaminophen, but his work of breathing increased. The child’s oxygen saturation on room air is 94%. What is the recommended first step in treatment for this child? b. Dexamethasone 6.6mg given IM

This child has an unrepaired structural heart lesion. What would you anticipate as first line pharmacologic therapy?

a. Diuretics b. Antibiotics c. Afterload reducer d. Milrinone

This child has an unrepaired structural heart lesion. What would you anticipate as first line pharmacologic therapy?

a. Diuretics

Which of the following is an appropriate antibiotic selection for a 2 month old infant presenting with URI symptoms, paroxysms of cough, and difficulty feeding?

a. Cefdinir b. Clindamycin c. Erythromycin d. Amoxicillin

Which of the following is an appropriate antibiotic selection for a 2 month old infant presenting with URI symptoms, paroxysms of cough, and difficulty feeding? c. Erythromycin

A child with a tracheostomy develops massive bright red bleeding from the tracheostomy tube. What are the important first steps in therapy?

A. Overdistention of tracheostomy tube cuff and preparation for OR B. Suction tracheostomy tube and increase PEEP on ventilator C. Change tracheostomy tube and deflate trach tube cuff D. Hand ventilate with room air and administer neosynephrine via tracheostomy tube

©202 0 Answer: A child with a tracheostomy develops massive bright red bleeding from the tracheostomy tube. What are the important first steps in therapy?

A. Overdistention of tracheostomy tube cuff and preparation for OR

What do you see?

a. Atelectasis b. Pleural effusion c. Pericardial effusion d. Pneumothorax

What do you see?

b. Pleural effusion

•Many respiratory infections and diseases are commonly encountered in pediatric patients •Improvement in diagnoses improves patient care and expedites appropriate management •May pediatric patients will require respiratory support, non‐invasive or invasive, while they recover from their disease