Respiratory Diseases of the Newborn Beth Mogensen, RRT-NPS 1 OBJECTIVES 1. 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 Development Week 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 closes 5 6 Fetal Lung Development Week 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 bronchi 7 Fetal Lung Histology STAGE 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 vascularized 8 Fetal Lung Histology STAGE 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 9 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 10 Respiratory Distress at Birth Rule 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 Newborn Transient 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 Newborn Respiratory 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 Cause… Maternal History Any risk factors Gestational age of Infant Amniotic fluid (color/odor/volume) Intrapartum history Clinical Presentation/ Assessment X-Rays Lab Evaluations 14 Clinical Presentation Respiratory Assessment Respiratory rate Quality Shallow Deep Nasal Flaring Grunting Retractions Breath Sounds 15 Clinical Presentation Color—pink, dusky, pale, mottled Central Peripherally Heart rate Pulses Distal vs Central Perfusion Capillary Refill Time (CRT) Blood Pressure 16 Clinical Presentation Physical characteristics Flat nasal bridge, Simian crease, recessed chin, low set ears Deformities Extra digits, gastroschesis, imperforate anus Muscular Hyoptonia vs Hypertonia Skeleton Choanal Atresia, Osteogenesis Imperfecta Other Scaphoid abdomen, heart tones on Right side 17 X-Ray Structures Ribs Vertebra Liver Stomach/ intestine Lungs Heart Trachea Esophagus 18 X-Ray Lungs Lung Volume Expansion Densities Fluid/ collapse (atelectasis)>>white Free Air>>dark Mass Heart shape and size Boot shaped Egg or Oval shaped 19 Lab Values CBC with diff ABG/CBG/VBG Blood Cultures CRP Electrolytes Type and Cross PKU 20 Respiratory Distress Determining Differential Diagnosis What you need to know… • • • • History Presentation/ clinical assessment X-rays Lab values 21 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 after 22 Transient Tachypnea of the Newborn History Common with C-Section delivery Maternal analgesia Maternal anesthesia during labor Maternal fluid administration Maternal asthma, diabetes, bleeding Perinatal asphyxia Prolapsed cord 23 TTN presents: Respiratory Assessment Tachypnea 60-150 bpm Nasal flaring Grunting Retracting Fine Rales Cyanotic 24 TTN X-Ray findings Prominent Perihilar streaking Hyperinflation Fluid in fissure Labs CBC within normal limits ABG/CBG showing mild to moderate hypercapnia, hypoxemia with a respiratory acidosis 25 TTN 26 TTN Have 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 distress 28 RDS History Gestational age < 38 weeks Prenatal care Diabetes (controlled vs uncontrolled) Perinatal infection Problems during pregnancy/delivery Asphyxia Stress to fetus Hypothyroidism Multiple births 29 RDS presents: Respiratory Assessment Tachypnea > 60 bpm Nasal flaring Grunting Retracting Apnea/ irregular respiratory pattern Rales (crackles) Diminished breath sounds Cyanosis 30 RDS X-Ray Loss of volume Reticulogranular pattern or “ground glass” appearance Air bronchograms Bell shaped thorax Air leak, PIE Loss of heart borders/ atelectasis White out 31 RDS 32 33 RDS 34 RDS Laboratory Results ABG/CBG Hypoxia Hypercarbia Acidosis CBC with Differential/ HHP Used to rule out other causes of respiratory distress Always check electrolytes, especially glucose, potassium and calcium 35 Treatment for RDS Post-Exogenous Surfactant Therapy Many on the market Prophylactic Treatment Administered in the delivery room Rescue Treatment Given after a definitive diagnosis of RDS 36 MECONIUM ASPIRATION SYNDROME Most 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 period 37 MAS History Prenatal Care Maternal diabetes Pregnancy Induced Hypertension (PIH) Pre-eclampsia Problems during pregnancy/delivery Color of amniotic fluid 38 MAS Respiratory Assessment Tachypnea Nasal flaring Grunting Retracting Apnea/ irregular respiratory pattern Decreased breath sounds/ wet/ rhonchi 39 MAS Clinical Assessment Color Pale/gray Cyanotic Stained skin X-Ray Increased AP diameter Hyperinflation Atelectasis Pneumothorax 40 MAS 41 MAS 42 Pneumonia/ Sepsis Occurs frequently in newborns 3 types Congenital Pneumonia Intrapartum Pneumonia Postnatal Pneumonia Most often seen with chorioamnionitis, prematurity and meconium aspiration Get thorough history 43 Causes Prematurity Prolonged rupture of membranes Maternal temp > 38C Foul smelling amniotic fluid Nonreassuring stress test Fetal tachycardia Meconium Maternal hx of STDs 44 Respiratory Assessment Tachypnea Apnea, irregular breathing pattern Grunting Retractions Nasal flaring Colorful secretions Rales, rhonchi Cyanosis 45 Clinical Assessment Gray, pale color Lethargy Temperature instability Skin rash-pettechia Tachycardia Glucose issues Hypoperfusion Oliguria 46 X-Ray Patchy infiltrates (aspiration) Bilateral diffuse granular pattern Streaky Loss of volume Densities 47 Pneumonia/ Sepsis 48 Pneumothorax and other Air Leaks History What happened in the delivery room? Was positive pressure given? Large amount of negative pressure generated with the 1st breath? 49 Pneumothorax/ Air Leaks Respiratory Assessment Tachypnea Nasal flaring Grunting Retractions BS absent or decreased 50 Pneumothorax/ Air Leak Clinical Assessment Cyanotic Pale, gray Heart Rate Tachycardia Bradycardia PEA Pulses Normal Poor absent 51 Pneumothorax/ Air Leak Perfusion Capillary Refill (CRT) Blood Pressure if monitoring Arterial Line, narrowing pulse pressure Deformities of Chest Wall Asymmetry of chest CHEST X-Ray speaks for itself!! 52 Pneumothorax 53 Pneumothorax Right lateral decubitus view of pneumothorax 54 Pneumopericardium 55 Congenital Abnormalities of the Lung and Thorax Congenital Heart Disease (CHD) Congenital Diaphragmatic Hernia (CDH) Congenital Cystic Adenomatiod Malformation Tracheal Abnormalities Esophageal Atresia Pulmonary Hypoplasia 56 Congenital Heart Disease Defect 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 pregnancy 57 CHD Two types of CHD Acyanotic-blood returning to Right side of heart passes thru lungs—usually defect in heart wall, or obstructed valve or artery Pink baby Sats within normal limits Cyanotic-have a mixing of oxygenated blood with venous blood—shunting ductus, PFO, ASD, VSD Blue baby Low sats 58 CHD Respiratory Assessment Respirations Normal 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 CHD Clinical Assessment HR Slow, fast, variable murmur BP Check in all 4 extremities Pulses in all extremities CRT in all 4 extremities Color Acyanotic -pink Cyanotic-blue 60 CHD Labs and Tests ABGs—dependent upon defect Lactic Acid Chest X-Ray Heart shape and size Pulmonary blood flow Echocardiogram Best test to aid in diagnosis Cardiac Cath for possible intervention 61 Congenital Diaphragmatic Hernia Congenital Cystic Adenomatoid Malformation Ideally 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 side 62 CDH 63 Congenital Diaphragmatic Hernia 64 CDH/ CCAM Respiratory Assessment Tachypneic Retractions Nasal flaring Grunting Breath Sounds Decreased on the affected side May hear bowel sounds in chest with CDH 65 CDH Clinical Assessment Scaphoid Abdomen- classic sign Color Cyanotic Heart Rate Fast, slow or normal Perfusion Depends upon the severity X-Ray—Best diagnostic tool Bowel, stomach, liver in chest ABGs Acidosis, 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 PPHN History Meconium? Asphyxia? Stress? Pneumonia/ Sepsis Primary Pulmonary Hypertension Dysfunction in pulmonary endothelial vasodilating mechanism CDH/ CCAM 71 PPHN Respiratory Assessment Tachypnea Retractions Grunting Nasal flaring Breath Sounds Depend on cause Pre and Post-ductal saturations to monitor shunting- best indicator if ECHO not available 72 PPHN Clinical Assessment Color Blue/ gray X-Ray Depends on cause Usually with decreased blood flow, minimal lung markings Lab Dependent on cause Many present with abnormal Platelets/ PT/ Fibrinogen ABG Respiratory and metabolic acidosis 73 Airway Abnormalities Occur 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 Abnormalities Supraglottic Nose-Choanal Atresia Craniofacial-Pierre Robin Macroglossia-Down’s Tumors-Hemangioma Glottic Vocal Cord Paralysis Tumors and Cysts Hemangioma, Cystic Hygroma, Teratoma Tracheal Esophageal Fistula/ Atresia Webs Trauma 75 Cystic Hygroma 76 Tracheal Esophageal Fistula/ Esophageal Atresia 77 Airway Abnormalities Subglottic Stenosis—congenital or acquired Webs Atresia Tumors Trachea Tracheomalacia Stenosis Cyst Atresia Extrinsic Vascular Ring Mediastinal Mass 78 History/ Presentation Circumstances 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 abnormalities 79 Airway Abnormalities Respiratory Assessment Tachypnea Retractions Work of Breathing Breath Sounds Stridor is the MOST important physical sign created by airway turbulence and indicates obstruction Inspiratory—implies supraglottic or glottic Expiratory—implies intrathoracic airway Mixed—implies subglottic 80 Airway Abnormalities Clinical Assessment Heart Rate Tachycardia Bradycardia when obstructed Color Cyanotic Lethargy Irritability Feeding Difficulty 81 Airway Abnormalities Bronchoscopy used for evaluating abnormality “Tools” for Treatment: Dependent upon Diagnosis Prone patient Oral Airway N-P Tube Steroids Meds for reflux OG, NG, NJ or G-Tube feedings 82 Airway Abnormalities Possible Surgical Interventions Cricoid Split Tracheostomy Excise Hygroma Place stents 83 Scenario Baby 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 Scenario Upon arriving in the mother’s room, you begin assessing infant. Baby’s color is rather blue. Tachypneic—RR 70s to 80s Bulb sx and get a little bit of colostrum. Retractions and Grunting present Baby’s temp is 35.9 What will you do? 85 Scenario 1. 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 80s—Retracting, nasal flaring, grunting HR 180—with murmur BP 42/30 with MAP 36 SaO2 on 100% blow by 88% 86 Scenario 5. Color—dusky 6. CRT 4 seconds 7. Poor peripheral pulses 87 Scenario CALL MD if you haven’t 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 Scenario When 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 Scenario STAT 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 transport… 90 Take Away… Don’t be afraid to “think out of the box” We continually see “funky” things Do no harm… this is someone’s baby 91 92 93