Respiratory Diseases of the Newborn

Beth Mogensen, RRT-NPS

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OBJECTIVESProvide overview of respiratory system of the newbornIdentify non-respiratory causes of distress in the newbornReview respiratory diseases/ anomalies of the newborn*

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Early Development*

Fetal Lung Development

Week 4: the laryngotracheal groove forms on the floor foregutWeek 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 closes*

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

Fetal Lung Histology

STAGE 1: Pseudoglandular Period (5-17 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 vascularized*

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 sacs*

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-ray *

Respiratory Distress at BirthRule of 6: non respiratory causes of distress

S & S Diagnosis ManagementHypothermia/ - check temperature - heat or cool as Hyperthermia necessaryHypovolemia - obtain prenatal history - gingerly give volumeHyoptension - measure blood pressure - give volume and/or vasopressorHypoglycemia - blood glucose measurement - give glucoseAnemia - measure hematocrit - transfuse with PRBC Polycythemia - measure hematocrit - partial exchange transfusion (lower Hct)

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

Respiratory Distress in the NewbornRespiratory CausesCongenital Abnormalities of the Lung/ThoraxCongenital Heart Disease (CHD)Congenital Diaphragmatic Hernia (CDH)Congenital Cystic Adenomatiod Malformation (CCAM)Tracheal AbnormalitiesEsophageal AtresiaPulmonary HypoplasiaPersistent Pulmonary Hypertension of the Newborn (PPHN)

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What do you need to knowto Figure out the CauseMaternal HistoryAny risk factorsGestational age of InfantAmniotic fluid (color/odor/volume)Intrapartum historyClinical Presentation/ AssessmentX-RaysLab Evaluations

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Clinical PresentationRespiratory AssessmentRespiratory rateQualityShallowDeepNasal FlaringGruntingRetractionsBreath Sounds

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Clinical PresentationColorpink, dusky, pale, mottledCentralPeripherallyHeart ratePulsesDistal vs CentralPerfusion Capillary Refill Time (CRT)Blood Pressure *

Clinical PresentationPhysical characteristicsFlat nasal bridge, Simian crease, recessed chin, low set earsDeformitiesExtra digits, gastroschesis, imperforate anusMuscular Hyoptonia vs HypertoniaSkeletonChoanal Atresia, Osteogenesis ImperfectaOtherScaphoid abdomen, heart tones on Right side*

X-RayStructuresRibsVertebraLiverStomach/ intestineLungsHeartTracheaEsophagus*

X-RayLungsLung VolumeExpansionDensitiesFluid/ collapse (atelectasis)>>whiteFree Air>>darkMassHeart shape and sizeBoot shapedEgg or Oval shaped*

Lab ValuesCBC with diffABG/CBG/VBGBlood CulturesCRPElectrolytesType and CrossPKU

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Respiratory DistressDetermining Differential Diagnosis

What you need to knowHistoryPresentation/ clinical assessmentX-raysLab values

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Transient Tachypnea of the Newborn (TTN)

Most common diagnosis of respiratory distress in the newbornRemember often term infants may be a little early Ineffective clearance of amniotic fluid from lungs with deliveryMost often seen at birth or shortly after

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Transient Tachypnea of the Newborn

HistoryCommon with C-Section deliveryMaternal analgesiaMaternal anesthesia during laborMaternal fluid administrationMaternal asthma, diabetes, bleedingPerinatal asphyxiaProlapsed cord*

TTN presents:Respiratory AssessmentTachypnea 60-150 bpmNasal flaringGruntingRetractingFine RalesCyanotic

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TTNX-Ray findingsProminent Perihilar streakingHyperinflationFluid in fissureLabsCBC within normal limitsABG/CBG showing mild to moderate hypercapnia, hypoxemia with a respiratory acidosis

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TTN*

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

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

RDSHistoryGestational age < 38 weeksPrenatal careDiabetes (controlled vs uncontrolled)Perinatal infectionProblems during pregnancy/deliveryAsphyxiaStress to fetus HypothyroidismMultiple births*

RDS presents:Respiratory AssessmentTachypnea > 60 bpmNasal flaringGruntingRetractingApnea/ irregular respiratory pattern Rales (crackles)Diminished breath soundsCyanosis

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RDSX-RayLoss of volumeReticulogranular pattern or ground glass appearanceAir bronchogramsBell shaped thoraxAir leak, PIELoss of heart borders/ atelectasisWhite out*

RDS*

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RDS*

RDSLaboratory ResultsABG/CBGHypoxiaHypercarbiaAcidosisCBC with Differential/ HHPUsed to rule out other causes of respiratory distressAlways check electrolytes, especially glucose, potassium and calcium*

Treatment for RDSPost-Exogenous Surfactant TherapyMany on the marketProphylactic Treatment Administered in the delivery roomRescue TreatmentGiven after a definitive diagnosis of RDS

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MECONIUM ASPIRATION SYNDROMEMost often found in post date infants > 40 weeks, but may occur in infants >34 weeksInfant passes meconium due to varying degrees of asphyxia in uteroObstruction of large and small airways with aspirated meconiumAspiration may occur: in utero intrapartumpostpartum period*

MASHistoryPrenatal CareMaternal diabetesPregnancy Induced Hypertension (PIH)Pre-eclampsiaProblems during pregnancy/deliveryColor of amniotic fluid*

MASRespiratory AssessmentTachypneaNasal flaringGruntingRetractingApnea/ irregular respiratory patternDecreased breath sounds/ wet/ rhonchi

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MASClinical AssessmentColorPale/grayCyanoticStained skinX-RayIncreased AP diameterHyperinflationAtelectasisPneumothorax*

MAS*

MAS*

Pneumonia/ SepsisOccurs frequently in newborns3 typesCongenital PneumoniaIntrapartum PneumoniaPostnatal PneumoniaMost often seen with chorioamnionitis, prematurity and meconium aspirationGet thorough history

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CausesPrematurityProlonged rupture of membranesMaternal temp > 38CFoul smelling amniotic fluidNonreassuring stress testFetal tachycardiaMeconiumMaternal hx of STDs*

Respiratory AssessmentTachypneaApnea, irregular breathing patternGruntingRetractionsNasal flaringColorful secretionsRales, rhonchiCyanosis*

Clinical AssessmentGray, pale colorLethargyTemperature instabilitySkin rash-pettechiaTachycardiaGlucose issuesHypoperfusionOliguria

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X-RayPatchy infiltrates (aspiration)Bilateral diffuse granular pattern StreakyLoss of volumeDensities*

Pneumonia/ Sepsis*

Pneumothorax and otherAir LeaksHistoryWhat happened in the delivery room?Was positive pressure given?Large amount of negative pressure generated with the 1st breath?*

Pneumothorax/ Air LeaksRespiratory AssessmentTachypneaNasal flaringGruntingRetractionsBS absent or decreased*

Pneumothorax/ Air LeakClinical AssessmentCyanoticPale, grayHeart RateTachycardiaBradycardiaPEAPulsesNormalPoorabsent*

Pneumothorax/ Air LeakPerfusionCapillary Refill (CRT)Blood Pressure if monitoring Arterial Line, narrowing pulse pressureDeformities of Chest WallAsymmetry of chest

CHEST X-Ray speaks for itself!!*

Pneumothorax*

PneumothoraxRight lateral decubitus view of pneumothorax*

Pneumopericardium*

Congenital Abnormalities of the Lung and ThoraxCongenital Heart Disease (CHD)Congenital Diaphragmatic Hernia (CDH)Congenital Cystic Adenomatiod MalformationTracheal AbnormalitiesEsophageal AtresiaPulmonary Hypoplasia*

Congenital Heart DiseaseDefect present at birth- often picked up on early ultrasoundIncreased risks:Parents have CHD?Siblings have CHD?Maternal diabetesExposure to German measles, toxoplasmosis, or if mother HIV+Alcohol use during pregnancyCocaine use during pregnancy*

CHDTwo types of CHDAcyanotic-blood returning to Right side of heart passes thru lungsusually defect in heart wall, or obstructed valve or arteryPink babySats within normal limitsCyanotic-have a mixing of oxygenated blood with venous bloodshunting ductus, PFO, ASD, VSDBlue babyLow sats

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

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CHDClinical AssessmentHR Slow, fast, variablemurmurBPCheck in all 4 extremitiesPulses in all extremitiesCRT in all 4 extremitiesColorAcyanotic -pinkCyanotic-blue*

CHDLabs and TestsABGsdependent upon defectLactic AcidChest X-RayHeart shape and sizePulmonary blood flowEchocardiogramBest test to aid in diagnosisCardiac Cath for possible intervention*

Congenital Diaphragmatic HerniaCongenital Cystic Adenomatoid MalformationIdeally diagnosed in uteroDevelops during pseudoglandular stage, but CCAM can form up to 35 weeks Normally compromised at delivery requiring immediate intubationCDH more commonly found on Left side

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CDH*

Congenital Diaphragmatic Hernia*

CDH/ CCAMRespiratory AssessmentTachypneicRetractionsNasal flaringGruntingBreath SoundsDecreased on the affected sideMay hear bowel sounds in chest with CDH*

CDHClinical AssessmentScaphoid Abdomen- classic signColorCyanoticHeart RateFast, slow or normalPerfusionDepends upon the severityX-RayBest diagnostic toolBowel, stomach, liver in chestABGsAcidosis, hypoxemia and hypercarbia*

Left Congenital DiaphragmaticHernia*

CCAM*

Persistent PulmonaryHypertension (PPHN)Pulmonary hypertension resulting in severe hypoxemia secondary to R>L shunt thru PFO and/or PDAUsually affecting term or near-term infantsMay be extremely difficult to manageIf not responding to available therapy consider transporting to an ECMO center

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Fetal Circulation*

PPHNHistoryMeconium?Asphyxia?Stress?Pneumonia/ SepsisPrimary Pulmonary HypertensionDysfunction in pulmonary endothelial vasodilating mechanismCDH/ CCAM*

PPHNRespiratory AssessmentTachypnea RetractionsGruntingNasal flaringBreath Sounds Depend on causePre and Post-ductal saturations to monitor shunting- best indicator if ECHO not available*

PPHNClinical AssessmentColorBlue/ grayX-RayDepends on causeUsually with decreased blood flow, minimal lung markingsLabDependent on causeMany present with abnormal Platelets/ PT/ FibrinogenABGRespiratory and metabolic acidosis

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

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Airway AbnormalitiesSupraglotticNose-Choanal AtresiaCraniofacial-Pierre RobinMacroglossia-DownsTumors-HemangiomaGlotticVocal Cord ParalysisTumors and CystsHemangioma, Cystic Hygroma, TeratomaTracheal Esophageal Fistula/ AtresiaWebsTrauma*

Cystic Hygroma*

Tracheal Esophageal Fistula/ Esophageal Atresia*

Airway AbnormalitiesSubglotticStenosiscongenital or acquiredWebsAtresiaTumorsTracheaTracheomalaciaStenosisCystAtresiaExtrinsicVascular RingMediastinal Mass*

History/ PresentationCircumstances surrounding onset of symptomsSpeed of progression of symptomsPosition of comfort and how change affects symptomsPresence of feeding abnormalitiesNature of cryPrevious infectionHistory of previous intubation or traumaPresence of associated cardiopulmonary abnormalities

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Airway AbnormalitiesRespiratory AssessmentTachypneaRetractionsWork of BreathingBreath SoundsStridor is the MOST important physical sign created by airway turbulence and indicates obstructionInspiratoryimplies supraglottic or glotticExpiratoryimplies intrathoracic airwayMixedimplies subglottic*

Airway AbnormalitiesClinical Assessment Heart RateTachycardiaBradycardia when obstructedColorCyanoticLethargyIrritabilityFeeding Difficulty*

Airway AbnormalitiesBronchoscopy used for evaluating abnormalityTools for Treatment:Dependent upon DiagnosisProne patientOral AirwayN-P TubeSteroidsMeds for refluxOG, NG, NJ or G-Tube feedings

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Airway AbnormalitiesPossible Surgical InterventionsCricoid SplitTracheostomyExcise HygromaPlace stents

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Scenario Baby Boy SNo 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.*

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

ScenarioTake infant back to nursery for observation and monitoring.Place infant on O2 if sat < 90-92 depending upon your policy.Place infant under radiant warmer.Obtain a full set of vital signs.RR 80sRetracting, nasal flaring, gruntingHR 180with murmurBP 42/30 with MAP 36SaO2 on 100% blow by 88%

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Scenario5. Colordusky6. CRT 4 seconds7. Poor peripheral pulses*

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

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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 bubblesWhat do you want to do?What do you suspect this infant has?*

ScenarioSTAT CXRIntubate infant and ventilatePlace large bore Anderson/ Replogle tube to continuous low suctionGive fluid bolus (and more if needed)Probably start pressorsDo what needs to be done to stabilize this infant and call for transport*

Take AwayDont be afraid to think out of the boxWe continually see funky thingsDo no harm this is someones baby*

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*****LT groove--forms on anterior wall of pharynx and forms larynx, trachea and respiratory tree***Pseudoglandularthe lungs look like an organ and NO respirations are possible!!CANALICULAR-by week 24 respiratory bronchioles have developed and respirations are possible*Surfactant reaches adequate levels 2 weeks before birth.****Mat hx: bleeding, fever, no pre-natal care, anything to give a heads up!! Diabetic, PIH,POLYHYDRAMNIOS--associated problems due to not swallowing amniotic fluid pulmonary hypoplasia, duodenal atresia, OLIGOHYDRAMNIOSlo fluid urio/kidney issuesGest age: preterm vs term and issues that go with it Intrapartum:(during L&D) Amniotic fluid-chorio, did mom receive drugs, poor tracing, infection OLIGOHYDRAMNIOSlo fluid urio/kidney issuesPOLYHYDRAMNIOS--Intrapartum-during labor and delivery; associated problems due to not swallowing amniotic fluid pulmonary hypoplasia, duodenal atresia,***When viewing an x-ray, best to get your routine down, always go in the same order to hopefully not miss anything*Often heart shape can give you a heads up on a type of CHD; boot shaped-TOF, egg-shaped- Transpositon of the Great Arteries (TGA)**Signs are usually seen within a few hours of delivery*Perihilararea around where lungs and heart meet, very vascular, lots of blood vesselsstreaking extra fluidFissure divides lobes of the lungs (3 on R, 2 on L)*REMEMBER before 24 weeks, no surfactant is producedSO, more premature you are, the less surfactant one will haveSurfactant like dish soap keeping alveoli from sticking together*DIABETES- too much insulin in babys system can delay surfactant productionHYPOTHYROIDISM-- not tx have an ^ incidence of preterm labor, PIH, fetal distress, IUGR, fetal anomaly, retardation*White out , no heart borders*Often given in the delivery room and infant extubated to CPAP shortly afterwards*OLIGOlow fluid

*Non-homogenous ---areas if hyperinflation *Areas of aeration and atelectasis*Aeration vs atelectasis*5-50 out of 1000 live birthsCONGENITAL before birthINTRAPARTUM- during deliveryPOSTNATAL-- within 24 hours after delivery

*OLIGURIAdecreased urine output 25*EXTRINSICGoiter, Hemangioma, Cystic Hygroma, Teratoma