PEDIATRIC AND NEONATAL DISEASES Upper Airway...

PEDIATRIC AND NEONATAL DISEASES

Upper Airway Diseases

Inc.

Pediatric Sleep-Disordered Breathing

Introduction

Sleep deprivation causes serious neurocognitive

deficits and may be lethal (indicated in studies with

animals).

Copyright © 2015, 2010, 2003, 1995 by Saunders, an imprint of

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Normal Sleep Development


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Normal newborn spends 70% of day asleep

3-4 hours of sleep

1-2 hours awake

Nonrapid eye movement (NREM)

Rapid eye movement (REM)

By 6 months of age

13-14 hours of sleep

6 to 8 hours in duration

Preschool age

11-12 hours of sleep

Disordered Breathing in Infants


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Periodic breathing

Common in infants

Rapid breathing followed by pauses > 3 seconds

Apnea

Respiratory pause > 15-20 seconds

Central apnea

No chest movement and no airflow

Obstructive apnea

Apnea of prematurity

Common in infants < 32 weeks’ gestation

Sudden Infant Death Syndrome (SIDS)

Leading cause of death in first 12 months of life

Third leading cause of death in infants

70% of victims found in early morning hours

Peak incidence―2 to 4 months of life

Uncommon after 6 months of life

Associated with prone position


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Acute Life-Threatening Event (ALTE)

Combination of

Apnea

Color change

Marked change in muscle tone

Choking or gagging


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Obstructive Sleep Apnea (OSA)

Complete or partial obstruction of the upper airway

Gas exchange and/or sleep are compromised

Major cause of OSA in children is adenotonsillar

hypertrophy

Peak prevalence: 28 years of age

Chronic obstruction = substantial morbidity

Daytime symptoms may include poor school

performance, hyperactivity


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Obstructive Sleep Apnea (Cont.)

Treatment options

Adenotonsillectomy

CPAP


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Laboratory Assessment of Breathing in

Sleep

Overnight polysomnography

ECG

Inductive plethysmography

Continuous esophageal pH measurement

Video recording with sound


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Upper Airway Disorders

Periglottic obstruction

Croup (laryngotracheobronchitis)

Epiglottitis

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Croup--Etiology

Viral Infection:

Parainfluenza

Influenza

RSV

Adenovirus

Gradual onset

Affects children 6 months to 3 years-old

Croup--Pathophysiology

Swelling and inflammation of subglottic structures.

Larynx

Trachea

Larger Bronchi

Can affect mid-sized and smaller airways

Croup--Clinical Manifestations

Slow onset, like a “cold”

Brassy/barking cough

Horseness & Audible stridor

Neck X-Ray: Steeple Sign

If Severe:

Tachycardia/tachypnea

Retractions

Decrease in SPO2

ABG: Hypoxemia & Respiratory Acidosis

Steeple Sign-Often Found in Croup

Croup--Treatment

Cool Mist w/oxygen via tent or face mask

Reassurance--Parental presence

Racemic Epinephrine via SVN or IPPB

6 Y.O. or less: 0.25 mls of 2.25% w/NSS

More than 6 Y.O. 0.5mls w/NSS

Systemic Steroids: Dextramethasone

0.3 to 0.6 mg/KG

Intubation: Mainly if respiratory failure present: e.g., muscle fatigue, change in sensorium, cyanosis,

ABG results.

Epiglottitis--Etiology

Bacterial infection

Most common microorganisms:

Staphylococcus Aureus

Group A & B Streptococci

Strep Pneumoniae

Other causes:

thermal injury

caustic ingestion

radiation exposure

Epiglottitis--Pathophysiology

Supra-glottic swelling

Epigottis turns bright, cherry red & swollen

Inflamation leads to a/w narrowing and dysphagia

If severe, a/w can become completely obstructed

Epiglottitis--Clinical Manifestations

Patient appears acutely ill

Rapid Onset

Affects mainly children 1 - 5 years old

Drooling, sore throat, dysphagia

Stridor & hoarseness w/diminished breath sounds in lung regions

High fever

Lateral neck x-ray: Balloon-shaped epiglottis/”thumb sign”

Lateral Neck X-Ray—Thumb Sign

Epiglottitis-Treatment

Minimal patient stimulation-keep patient calm!

Cool mist aerosol w/supp’l O2

Antibiotics and fluids (steroids generally not effective)

If severe obstruction, intubation shouldn’t be

attempted in ER

Intubate patient in OR as trach may be necessary

and patient may need to be paralyzed

Epiglottitis Vs. Croup

Epiglottitis

Bacterial

Rapid On-set

Profound illness

Hospitalization common required.

Pt. may be drooling and leaning forward with compromised speech

May need emergent care and airway management.

Croup

Viral

More gradual onset

Mild to moderate illness

Occasionally requires hospitalization

Mainly supportive care

FBAO

March

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PEDIATRIC RESUSCITATION I 22

Signs:

SUDDEN onset of respiratory distress

Weak or silent coughing

Inability to speak

Stridor

Increasing respiratory difficulty

Cyanosis

FBAO (cont.)

March

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PEDIATRIC RESUSCITATION I 23

Infant: Back blows-chest thrust (5:5)

Child: Abdominal thrust and Heimlich maneuver

DO NOT perform finger sweep in a responsive

victim

Blind finger sweeps should not be perform in

infants

Pneumonia

Viral

Respiratory syncytial virus (RSV)

Parainfluenza virus types 1, 2, and 3

Influenza viruses

Adenovirus


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Pneumonia (Cont.)

Bacterial

Etiology

Viral or bacterial

Signs and symptoms

Resp. Distress: Retractions, nasal flaring, Inc RR

Diagnosis

CXR

Sputum C&S

Treatment

Supportive, ABX


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Asthma


Pathogenesis of Asthma


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Definition

PathophysiologyChronic airway inflammation

Bronchial hyperresponsiveness

Hypersecretion of mucus

Risk Factors for Developing

Asthma


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Allergic response Atopy—strongest predisposing factor for

developing asthma

Atopic dermatitis

Environmental triggers Tobacco smoke

Cockroach antigens

Molds

House dust mites

Diagnosis


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Medical history

Physical examination

Pulmonary function testing

Exhaled nitric oxide

Bronchoprovocational challengesMethacholine

Exercise

Differential diagnosis

Management of Asthma


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Pharmacologic therapy Long-term control medications

Quick-relief medications

Delivery systems

Control of asthma triggers Identification of allergens

Avoidance and control measures

Role of immunotherapy

Management of Asthma (Cont.)


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Peak flow monitoringPeak flow meter

Peak flow diary

Personal best reading

Peak flow zone system



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Asthma action planPlan when symptomatic

Based on current peak flow reading and personal

best number

Actions Begin quick-relief medications.

Increase daily medications.

Contact the physician.

Seek emergency care.



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Patient and family education Asthma disease process

Medication skills

Identification and control of triggers

Self-monitoring techniques

Managing Asthma Exacerbations in

the ED


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Assessment

Beta2 agonists

Corticosteroids

Hospitalization and Respiratory

Failure


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Intubation

Semielective vs. emergent

Mechanical ventilation

VT = 6-8 mL/kg

Low to moderate PEEP

Permissive hypercapnia

Exercise-Induced Bronchospasm

(EIB)


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Peaks 5 to 10 minutes after cessation of

activity

May resolve spontaneously in 20 to 30 minutes

Hyperventilation of cool and dry air

Inhalation of beta2 agonist, cromolyn,

nedocromil, or salmeterol 5 to 60 minutes

before exercise

5- to 10-minute warm-up

Asthma at School


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Leading cause of absenteeismMore than 10 days/year

Poor academic achievement

Inability to participate in school activities

Low self-esteem

Peak Performance USA

Cystic Fibrosis


Introduction


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Genetic disorder

Cystic fibrosis transmembrane conductance

regulator (CFTR) dysfunction

Complications

Thick, viscous secretions

Chronic obstruction, infection, inflammation of airways

Exocrine pancreatic insufficiency

Infertility in males

Elevated sweat chloride levels

Epidemiology


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Most common genetic disease among whites

One in 28 are carriers.

Median age at diagnosis: 5 months

Genetics and Molecular Biology


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Chromosome 7

Alteration of sodium and chloride transport

Autosomal recessive

Each parent is an obligate carrier.

Diagnosis


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Sweat chloride testing

Gold standard for diagnosis

>60 mmol/L—confirmatory

40-59 mmol/L—intermediate

CFTR mutation analysis

Newborn screening

Pulmonary Disease


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Mucus production and airway obstruction

Bacterial infection

Pseudomonas aeruginosa

Burkholderia cepacia

Airway inflammation

Clinical manifestations

Treatment of Pulmonary Disease


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Aerosol therapy

Airway clearance therapy

Antibiotics

Anti-inflammatory agents

Macrolides

Corticosteroids

Ibuprofen

Lung transplantation

Small-molecule CFTR modulators

Nonpulmonary Manifestations


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Upper airway disorders

Gastrointestinal disorders

Pancreatic insufficiency

Meconium ileus and distal intestinal obstruction

syndrome

Rectal prolapse

Gastroesophageal reflux disease

Hepatobiliary disorders

Prognosis


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Improving outcomes

Median predicted age of survival: 38.3 years

Acute Respiratory Distress Syndrome

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Inc.

Acute Respiratory Distress Syndrome

(ARDS)

AECC definition

Acute onset of respiratory symptoms

Frontal chest radiograph with bilateral infiltrates

Pao2/Fio2 ≤ 200 mm Hg

No clinical evidence of left atrial hypertension as

defined by a pulmonary artery wedge pressure ≤ 18

mm Hg, if measured


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ARDS (Cont.)

Etiology

Usually direct resp. insult

Distant site

Iatrogenic

Sepsis

Clinical course

Stages of ARDS

Mortality: < 20% with early intervention


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Pathology and Role of Inflammatory

Mediators

Pathology and pathophysiology

Exudative stage

Proliferative stage

Fibrotic stage

Inflammatory mediators


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Treatment and Ventilatory Support

Goals

Achieve adequate tissue oxygenation.

Avoid complications.

PEEP

Low tidal volume ventilation

Gas exchange goals

Permissive hypercapnia


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Adjunctive Therapies

HFV

ECMO


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Shock

Disruption of natural homeostasis

Inability to maintain sufficient supply of oxygen

Potentially fatal when untreated

Cardiac output is impaired


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Definition and Classification

Hypovolemic

Most common

Cardiogenic

Obstructive

Physical obstruction to blood flow

Cardiac tamponade, tension pneumothorax, pulmonary embolism

Distributive

Sepsis

Anaphylaxis


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Pathophysiology


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Cardiac output

Stroke volume

Nutrients and oxygen in blood

Oxygen delivery

Delivery of blood to the tissues

Metabolic response

Management and Treatment


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General considerations

Assessment and evaluation

Monitoring

Central venous pressure (CVP)

Pulmonary artery (PA) catheter

Management and Treatment (Cont.)


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System-based treatment approach

Respiratory

Vascular volume

Myocardial function

Hematologic

Endocrine

Immunologic

Nutritional

ECMO

Pediatric Trauma

Inc.

Overview


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Epidemiology

Anatomic considerations

Size and shape differences

Mechanisms of injury

Initial assessment and diagnosis

Airway patency

Breathing support

Circulation

Disability

Thoracic Trauma


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Birth trauma

Nerve injury

Congenital chylothorax

Pneumothorax

Blunt thoracic trauma

Pulmonary contusion

Flail chest

Penetrating thoracic trauma

Iatrogenic thoracic trauma

Thoracic Trauma (Cont.)


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Penetrating thoracic trauma

Incidence

Resuscitation

Imaging

Iatrogenic thoracic trauma

Pneumomediastinum

Pneumothorax

Esophageal-pharyngeal injuries

Ventilator-induced injuries

Thermal Injury


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Epidemiology

Scald injuries: most common (0-4 years)

High-mortality risk factors

Pathophysiology

Classification of burn injury

Percent of body surface area burn

Management

Long-term outcomes

Inhalation Injury


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Pathophysiology

Upper airway injury

Lung parenchyma injury

CO poisoning

Evaluation of injury


Bronchoscopy

Xenon scan

Spirometry

Thermal and dye dilution

Inhalation Injury (Cont.)


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Management

Oxygen therapy

Airway maintenance

Bronchial hygiene therapy

Pharmacologic management

Mechanical ventilatory support

Conventional mechanical ventilation

High-frequency percussive ventilation

Complications

Long-term outcomes

Drowning


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Incidence

One in four fatal drowning victims <14 years of age

Open water is most common site

Witnessed in < 20% of younger children drowning

Pathophysiology

Central nervous system effects

Pulmonary effects

Cardiovascular effects

Drowning (Cont.)


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Treatment

At the scene

Emergency department

Inpatient

Outcome

Prevention

Neurological and Neuromuscular

Disorders

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Inc.

Neuromuscular Control of Ventilation

Central nervous system

Peripheral nervous system

Respiratory muscles

Respiratory control system

Bulbar muscles


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Neuromuscular Diseases That Affect the

Respiratory System

Central nervous system

Disorders of the brain

Disorders affecting the spinal cord

Trauma

Chiari malformations

Other conditions


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Respiratory System (Cont.)

Peripheral nervous system

Disorders of the motor nerves

Spinal muscle atrophy

Poliomyelitis

Guillain-Barré syndrome


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Peripheral nervous system (Cont.)

Disorders of the neuromuscular junction

Infantile botulism

Myasthenia gravis

Other


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Myopathies

Duchenne and Becker muscular dystrophy

Myotonic dystrophies

Glycogen storage diseases

Electrolyte abnormalities


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Respiratory Evaluation of Children

with Neuromuscular Disease

PFT (serial)

Spirometry

CO2 and O2 analysis

Cough effectiveness and lung volume

ETCO2

Sleep studies


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Respiratory Care of Children with

Neuromuscular Disease

General considerations

Airway clearance mechanisms

Facilitating mucus clearance

Assisted coughing

Glossopharyngeal breathing


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Respiratory Care of Children with

Neuromuscular Disease (Cont.)

Mechanical ventilatory support

Noninvasive ventilation

Nocturnal ventilation

Tracheostomy


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Neurologic and Neuromuscular

Disorders

Nonrespiratory care

Nutritional

Cardiac status

Extent of scoliosis

Surgical corrections

End-of-life issues


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Neonatal Pulmonary Disorders


Respiratory Distress Syndrome


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Incidence– 1% of pregnancies– 60%-80% in infants < 28 weeks

Cause and pathophysiology– Role of surfactant

Clinical presentation and diagnosis– Tachypnea– Grunt– Nasal flaring– Retractions – Cyanosis – L/S ratio (>2.0)

Respiratory Distress Syndrome

(Cont.)


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Prevention Avoid poorly timed cesarean section.

Antenatal and intrapartum fetal monitoring

Antenatal steroid therapy

Treatment Surfactant

Oxygen therapy

CPAP


Complications and prognosis

Bronchopulmonary Dysplasia


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Definition

Incidence

Pathophysiology and risk factors Prematurity

Ventilator-induced lung injury

Hypoxia- or hyperoxia-induced lung injury

Inflammation

Nutrition

PDA

Genetics

Vascular



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Clinical presentation and diagnosis

Differential diagnosis

Prevention Lung protective ventilator strategies

Oxygen therapy

Antioxidants

Inhaled NO

Nutrition

Corticosteroids

Methylxanthines


(Cont.)


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Treatment

Oxygenation


Fluid management

Drug therapies

Nutrition

Social issues

Complications and prognosis

Surgical Disorders in Childhood

that Affect Respiratory Care


Upper Airway Conditions


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Choanal atresiaObligate nasal breathers

Unilateral is most common

Bilateral is neonatal emergency

Oral airway

Macroglossia Beckwith-Wiedemann and Down syndrome

Mandibular hypoplasia Pierre Robin or Treacher Collins syndrome

Esophageal Atresia and

Tracheoesophageal Fistula


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Symptoms

Diagnosis

Treatment

Complications Tracheomalacia

Management Preoperative

Perioperative

Postoperative

Chest Wall Malformations


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Pectus excavatum

Pectus carinatum

Scoliosis and kyphoscoliosis

Congenital Cardiac Defects

Shunt Lesions


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Patent ductus arteriosus (PDA)

48 hours after birth

Decrease in PgE2 + increase in Pao2 = closure

Predisposing factors for PDA

Prematurity (most common)

Persistent pulmonary hypertension

Respiratory distress syndrome (RDS)

Clinical signs

Management

Patent ductus arteriosus (PDA)


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Shunt Lesions (Cont.)


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Atrial septal defect (ASD)

Left-to-right shunting

Only 8% of children with ASD develop symptoms

before 2 years of age.

Surgical repair



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Ventricular septal defect (VSD)

Most common congenital heart defect


Eisenmenger’s syndrome

Occurs when pulmonary vascular resistance nears that of the

systemic vascular resistance

Left-to-right shunt may reverse to right-to-left shunt

Therapy



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Atrioventricular septal defect (AVSD)

AV canal or endocardial cushion defect


Most common congenital heart lesion in infants with

Down syndrome (trisomy 21)


Surgical repair

Left Ventricular Outflow Tract

Obstruction


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Aortic stenosis (AS)

Valvular is most common type

Clinical presentation

Emergency therapy

Prostaglandin E1

Surgical repair

Left Ventricular Outflow Tract

Obstruction (Cont.)


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Coarctation of the aorta

Increase LV afterload


Treatment



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Other Cyanotic Congenital Heart

Lesions


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Tetralogy of Fallot (TOF)

Truncus arteriosus

Transposition of the great arteries (TGA)

Tetralogy of Fallot (TOF)

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Transposition of the great arteries

(TGA)


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Clinical Monitoring of Patients with

Cardiac Anomalies


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Hemodynamic monitoring

Pulse oximetry

Capnography

Cardiac output monitoring

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