Respiratory Diseases of the Newborn

Beth Mogensen, RRT-NPS1

OBJECTIVES1. Provide overview of respiratory system of the newborn 2. Identify non-respiratory causes of distress in the newborn 3. Review respiratory diseases/ anomalies of the newborn

2

3

Early Development

4

Fetal Lung DevelopmentWeek 4: the laryngotracheal groove forms on the floor foregut Week 5: the left and right lung buds push into the pericardioperitoneal canals (primordial of pleural cavity) Week 6: the descent of heart and lungs into the thorax. Pleuroperitoneal foramen closes5

6

Fetal Lung DevelopmentWeek 7: the lung buds divide into secondary and tertiary bronchi Week 24: the bronchi divide 14 more times and the respiratory bronchioles develop By birth, there will be an additional 7 divisions of bronchi7

Fetal Lung HistologySTAGE 1: Pseudoglandular Period (517 weeks) all the major elements of the lungs have formed except for those involved with gas exchange STAGE 2: Canalicular Period (16-25 weeks) bronchi and terminal bronchioles increase in lumen size and the lungs become vascularized8

Fetal Lung HistologySTAGE 3: Terminal Sac Period (24 weeks to birth) more terminal sacs develop and interface with capillaries lined with Type I alveolar cells or pneumocytes --Also have Type II pneumocytes which secrete surfactant thereby decreasing the surface tension forces and aids in expansion of the terminal sacs9

STAGE 4: Alveolar Period (late fetal period to 8 years) 95% of mature alveoli develop after birth. A newborn has only 1/6 to 1/8 of the adult number of alveoli and lungs appear denser on x-ray10

Respiratory Distress at BirthRule of 6: non respiratory causes of distress S&S Hypothermia/ Hyperthermia Hypovolemia Hyoptension Hypoglycemia Anemia Polycythemia transfusion

Diagnosis- check temperature - obtain prenatal history - measure blood pressure - blood glucose measurement - measure hematocrit - measure hematocrit

Management- heat or cool as necessary - gingerly give volume - give volume and/or vasopressor - give glucose - transfuse with PRBC - partial exchange (lower Hct)11

Respiratory Distress in the NewbornTransient Tachypnea of the Newborn (TTN) Surfactant Deficiency (HMD,RDS) Meconium Aspiration Syndrome (MAS) Pneumonia/ Sepsis Pneumothorax or other air leaks

12

Respiratory Distress in the NewbornRespiratory Causes Congenital Abnormalities of the Lung/Thorax

Congenital Heart Disease (CHD) Congenital Diaphragmatic Hernia (CDH) Congenital Cystic Adenomatiod Malformation (CCAM) Tracheal Abnormalities Esophageal Atresia Pulmonary Hypoplasia

Persistent Pulmonary Hypertension of the Newborn (PPHN)13

What do you need to know to Figure out the CauseMaternal History

Any risk factors

Gestational age of Infant Amniotic fluid (color/odor/volume) Intrapartum history Clinical Presentation/ Assessment X-Rays Lab Evaluations14

Clinical PresentationRespiratory Assessment

Respiratory rate QualityShallow Deep

Nasal Flaring Grunting Retractions Breath Sounds15

Clinical PresentationColorpink, dusky, pale, mottled

Central Peripherally

Heart rate Pulses

Distal vs Central Capillary Refill Time (CRT) Blood Pressure16

Perfusion

Clinical PresentationPhysical characteristics

Flat nasal bridge, Simian crease, recessed chin, low set ears Extra digits, gastroschesis, imperforate anus Hyoptonia vs Hypertonia Choanal Atresia, Osteogenesis Imperfecta Scaphoid abdomen, heart tones on Right side17

Deformities

Muscular

Skeleton

Other

X-RayStructures

Ribs Vertebra Liver Stomach/ intestine Lungs Heart Trachea Esophagus18

X-RayLungs

Lung Volume Expansion DensitiesFluid/ collapse (atelectasis)>>white Free Air>>dark Mass

Heart shape and size

Boot shaped Egg or Oval shaped

19

Lab ValuesCBC with diff ABG/CBG/VBG Blood Cultures CRP Electrolytes Type and Cross PKU20

Respiratory DistressDetermining Differential Diagnosis

What you need to know History Presentation/ clinical assessment X-rays Lab values21

Transient Tachypnea of the Newborn (TTN)Most common diagnosis of respiratory distress in the newborn Remember often term infants may be a little early Ineffective clearance of amniotic fluid from lungs with delivery Most often seen at birth or shortly after22

Transient Tachypnea of the NewbornHistory

Common with C-Section delivery Maternal analgesia Maternal anesthesia during labor Maternal fluid administration Maternal asthma, diabetes, bleeding Perinatal asphyxia Prolapsed cord23

TTN presents:Respiratory Assessment

Tachypnea 60-150 bpm Nasal flaring Grunting Retracting Fine Rales Cyanotic24

TTNX-Ray findings

Prominent Perihilar streaking Hyperinflation Fluid in fissure CBC within normal limits ABG/CBG showing mild to moderate hypercapnia, hypoxemia with a respiratory acidosis25

Labs

TTN

26

TTNHave delayed reabsorption of fetal lung fluid which eventually will clear over several hours to days Treatment: Treat signs and symptoms. Support infant, may need O2, is probably too tachypneic to PO feed so start IV fluids Be patient!!27

Surfactant Deficiency (RDS, HMD)One of the most common problems associated with a premature infant Decreased surfactant production in lungs of pre-term infants With decreased surfactant production, alveoli collapse, become atelectatic, yielding poor lung function and increasing signs of respiratory distress28

RDSHistory

Gestational age < 38 weeks Prenatal careDiabetes (controlled vs uncontrolled) Perinatal infection

Problems during pregnancy/deliveryAsphyxia Stress to fetus Hypothyroidism

Multiple births29

RDS presents:Respiratory Assessment

Tachypnea > 60 bpm Nasal flaring Grunting Retracting Apnea/ irregular respiratory pattern Rales (crackles) Diminished breath sounds Cyanosis30

RDSX-Ray

Loss of volume Reticulogranular pattern or ground glass appearance Air bronchograms Bell shaped thorax Air leak, PIE Loss of heart borders/ atelectasis White out31

RDS

32

33

RDS

34

RDSLaboratory Results

ABG/CBGHypoxia Hypercarbia Acidosis

CBC with Differential/ HHPUsed to rule out other causes of respiratory distress

Always check electrolytes, especially glucose, potassium and calcium35

Treatment for RDSPost-Exogenous Surfactant Therapy

Many on the marketProphylactic Treatment

Administered in the delivery room Given after a definitive diagnosis of RDS

Rescue Treatment

36

MECONIUM ASPIRATION SYNDROMEMost often found in post date infants > 40 weeks, but may occur in infants >34 weeks Infant passes meconium due to varying degrees of asphyxia in utero Obstruction of large and small airways with aspirated meconium Aspiration may occur:

in utero intrapartum postpartum period37

MASHistory Prenatal CareMaternal diabetes Pregnancy Induced Hypertension (PIH) Pre-eclampsia

Problems during pregnancy/delivery Color of amniotic fluid

38

MASRespiratory Assessment

Tachypnea Nasal flaring Grunting Retracting Apnea/ irregular respiratory pattern Decreased breath sounds/ wet/ rhonchi

39

MASClinical Assessment

ColorPale/gray Cyanotic Stained skin

X-Ray

Increased AP diameter Hyperinflation Atelectasis Pneumothorax40

MAS

41

MAS

42

Pneumonia/ SepsisOccurs frequently in newborns 3 types

Congenital Pneumonia Intrapartum Pneumonia Postnatal Pneumonia

Most often seen with chorioamnionitis, prematurity and meconium aspiration Get thorough history43

CausesPrematurity Prolonged rupture of membranes Maternal temp > 38C Foul smelling amniotic fluid Nonreassuring stress test Fetal tachycardia Meconium Maternal hx of STDs44

Respiratory AssessmentTachypnea Apnea, irregular breathing pattern Grunting Retractions Nasal flaring Colorful secretions Rales, rhonchi Cyanosis45

Clinical AssessmentGray, pale color Lethargy Temperature instability Skin rash-pettechia Tachycardia Glucose issues Hypoperfusion Oliguria46

X-RayPatchy infiltrates (aspiration) Bilateral diffuse granular pattern Streaky Loss of volume Densities

47

Pneumonia/ Sepsis

48

Pneumothorax and other Air LeaksHistory

What happened in the delivery room? Was positive pressure given? Large amount of negative pressure generated with the 1st breath?

49

Pneumothorax/ Air LeaksRespiratory Assessment

Tachypnea Nasal flaring Grunting Retractions BS absent or decreased

50

Pneumothorax/ Air LeakClinical Assessment

Cyanotic Pale, gray Heart RateTachycardia Bradycardia PEA

PulsesNormal Poor absent51

Pneumothorax/ Air LeakPerfusion

Capillary Refill (CRT) Blood Pressure if monitoring Arterial Line, narrowing pulse pressure Asymmetry of chest

Deformities of Chest Wall

CHEST X-Ray speaks for itself!!52

Pneumothorax

53

PneumothoraxRight lateral decubitus view of pneumothorax

54

Pneumopericardium

55

Congenital Abnormalities of the Lung and ThoraxCongenital Heart Disease (CHD) Congenital Diaphragmatic Hernia (CDH) Congenital Cystic Adenomatiod Malformation Tracheal Abnormalities Esophageal Atresia Pulmonary Hypoplasia56

Congenital Heart DiseaseDefect present at birth- often picked up on early ultrasound Increased risks:

Parents have CHD? Siblings have CHD? Maternal diabetes Exposure to German measles, toxoplasmosis, or if mother HIV+ Alcohol use during pregnancy Cocaine use during pregnancy57

CHDTwo types of CHD

Acyanotic-blood returning to Right side of heart passes thru lungsusually defect in heart wall, or obstructed valve or arteryPink baby Sats within normal limits

Cyanotic-have a mixing of oxygenated blood with venous bloodshunting ductus, PFO, ASD, VSDBlue baby Low sats58

CHDRespiratory Assessment

RespirationsNormal Tachypnea

Saturations depend upon defect.Acyanotic lesions sats are more normal Cyanotic lesions acceptable sats are low ~ 70% is acceptable; ideally on 21% FiO2

59

CHDClinical Assessment

HRSlow, fast, variable murmur

BPCheck in all 4 extremities

Pulses in all extremities CRT in all 4 extremities ColorAcyanotic -pink Cyanotic-blue60

CHDLabs and Tests

ABGsdependent upon defect Lactic Acid Heart shape and size Pulmonary blood flow Best test to aid in diagnosis

Chest X-Ray

Echocardiogram

Cardiac Cath for possible intervention61

Congenital Diaphragmatic Hernia Congenital Cystic Adenomatoid MalformationIdeally diagnosed in utero Develops during pseudoglandular stage, but CCAM can form up to 35 weeks Normally compromised at delivery requiring immediate intubation CDH more commonly found on Left side62

CDH

63

Congenital Diaphragmatic Hernia

64

CDH/ CCAMRespiratory Assessment

Tachypneic Retractions Nasal flaring Grunting Breath SoundsDecreased on the affected side May hear bowel sounds in chest with CDH

65

CDHClinical Assessment Clin

Scaphoid Abdomen- classic sign ColorCyanotic

Heart RateFast, slow or normal

PerfusionDepends upon the severity

X-RayBest diagnostic toolBowel, stomach, liver in chest

ABGsAcidosis, hypoxemia and hypercarbia

66

Left Congenital Diaphragmatic Hernia

67

CCAM

68

Persistent Pulmonary Hypertension (PPHN)Pulmonary hypertension resulting in severe hypoxemia secondary to R>L shunt thru PFO and/or PDA Usually affecting term or near-term infants May be extremely difficult to manage If not responding to available therapy consider transporting to an ECMO center

69

Fetal Circulation

70

PPHNHistory

Meconium? Asphyxia? Stress? Pneumonia/ Sepsis Primary Pulmonary HypertensionDysfunction in pulmonary endothelial vasodilating mechanism

CDH/ CCAM71

PPHNRespiratory Assessment

Tachypnea Retractions Grunting Nasal flaring Breath SoundsDepend on cause

Pre and Post-ductal saturations to monitor shunting- best indicator if ECHO not available72

PPHNClinical Assessment

ColorBlue/ gray

X-RayDepends on cause Usually with decreased blood flow, minimal lung markings

LabDependent on cause Many present with abnormal Platelets/ PT/ Fibrinogen

ABGRespiratory and metabolic acidosis73

Airway AbnormalitiesOccur less frequently than pulmonary parenchymal diseases Presentation is often quite dramatic with significant respiratory distress Stridor may be an important key to diagnosing the abnormality

74

Airway AbnormalitiesSupraglottic

Nose-Choanal Atresia Craniofacial-Pierre Robin Macroglossia-Downs Tumors-Hemangioma Vocal Cord Paralysis Tumors and CystsHemangioma, Cystic Hygroma, Teratoma Tracheal Esophageal Fistula/ Atresia Webs Trauma75

Glottic

Cystic Hygroma

76

Tracheal Esophageal Fistula/ Esophageal Atresia

77

Airway AbnormalitiesSubglottic

Stenosiscongenital or acquired Webs Atresia Tumors Tracheomalacia Stenosis Cyst Atresia Vascular Ring Mediastinal Mass

Trachea

Extrinsic

78

History/ Presentation PCircumstances surrounding onset of symptoms Speed of progression of symptoms Position of comfort and how change affects symptoms Presence of feeding abnormalities Nature of cry Previous infection History of previous intubation or trauma Presence of associated cardiopulmonary abnormalities79

Airway AbnormalitiesRespiratory Assessment

Tachypnea Retractions Work of Breathing Stridor is the MOST important physical sign created by airway turbulence and indicates obstructionInspiratoryimplies supraglottic or glottic Expiratoryimplies intrathoracic airway Mixedimplies subglottic

Breath Sounds

80

Airway AbnormalitiesClinical Assessment

Heart RateTachycardia Bradycardia when obstructed

ColorCyanotic

Lethargy Irritability Feeding Difficulty81

Airway AbnormalitiesBronchoscopy used for evaluating abnormality Tools for Treatment:

Dependent upon DiagnosisProne patient Oral Airway N-P Tube Steroids Meds for reflux OG, NG, NJ or G-Tube feedings82

Airway Abnormalities

Possible Surgical InterventionsCricoid Split Tracheostomy Excise Hygroma Place stents

83

ScenarioBaby Boy S

No prenatal care. Uneventful delivery vaginal delivery. APGARS 8 and 9 at 1 and 5 minutes, respectively. Infant taken to newborn nursery and given routine care. Eyes and thighs done, bath completed. VSS. Looking good and smelling nice. Infant went out to mother to breast feed and you have been summoned to check on baby. 84

ScenarioUpon arriving in the mothers room, you begin assessing infant. Babys color is rather blue. TachypneicRR 70s to 80s Bulb sx and get a little bit of colostrum. Retractions and Grunting present Babys temp is 35.9 What will you do?85

Scenario1. Take infant back to nursery for observation and monitoring. 2. Place infant on O2 if sat < 90-92 depending upon your policy. 3. Place infant under radiant warmer. 4. Obtain a full set of vital signs.1. 2. 3. 4.

RR 80sRetracting, nasal flaring, grunting HR 180with murmur BP 42/30 with MAP 36 SaO2 on 100% blow by 88%86

Scenario

5. 6. 7.

Colordusky CRT 4 seconds Poor peripheral pulses

87

ScenarioCALL MD if you havent already Continually reassess infant. With your next assessment: VS have not changed much. Infant continues to grunt, retract and have nasal flaring. Sats 86.

88

ScenarioWhen auscultating, you notice that the heart tones are now more midline than on left. You also notice that when auscultating the left lung that you thought you heard gas bubbles What do you want to do? What do you suspect this infant has?

89

ScenarioSTAT CXR Intubate infant and ventilate Place large bore Anderson/ Replogle tube to continuous low suction Give fluid bolus (and more if needed) Probably start pressors Do what needs to be done to stabilize this infant and call for transport90

Take AwayDont be afraid to think out of the box We continually see funky things Do no harm this is someones baby

91

92

93