LMCC Review Course “Neonatology” Brigitte Lemyre, MD, FRCPC

Outline Resuscitation principles, transition to life Normal newborn care and assessment IUGR and LGA and their problems Prematurity and its complications Problems of the term infant

Infant mortality: Normal baby at term: 9-10: 1000 births Due to congenital anomalies, prematurity, asphyxia, infections, SIDS Normal baby at term: HR: 120-160/min RR: 40-60/min Weight: 2.5-4.5 kg BP: 50-80/30-40 mmHg

Gestational age and size 28 weeks 1.0 kg 30 weeks 1.5 kg 33 weeks 2.0 kg 35 weeks 2.3 kg 37-40 weeks 2.5 – 4.5 kg

Newborn Resuscitation Initial steps Evaluate respiration Evaluate heart rate Evaluate color Remember - the usual problem in the neonate is the lungs: VENTILATION!

Fluid filled alveoli in utero Diminished blood flow through fetal lungs

Importance of first breath

Newborn Resuscitation A: Airway B: Breathing C: Circulation D: Drugs E: Environment F: Fluids G: Glucose

Special Circumstances in Newborn Resuscitation Meconium in amniotic fluid AND depressed newborn (not crying, limp): Intubate and suction below cords Suspect diaphragmatic hernia: Intubate Pink when crying, blue when not: Suspect choanal atresia and try an oral airway

The Apgar Score

Principles of Routine Care Ensure warmth and adequate nutrient intake Monitor weight, hydration status Support breastfeeding Educate about infant care Anticipatory guidance

Principles of Routine Care Prophylaxis for common problems Eye care: erythromycin ointment Vitamin K: 1 mg IM Screening for disease: >24h PKU (1/15,000) Hypothyroidism (1/4000) Neurosensory hearing loss 24 other metabolic diseases (organic acid disorders, FAOD, aminoacid disorders, sickle cell and hemoglobinopathies, CAH, galactosemia, endocrinopathies) Blood group and Coombs if mother rH neg

The depressed newborn Asphyxia Respiratory condition Hypovolemia/shock Drugs CNS Trauma Congenital malformations

Perinatal Asphyxia Must be documented by cordocentesis, fetal scalp blood sampling, cord blood sampling pH < 7.00, base deficit > 15 mEq/L Encephalopathy Multiorgan involvement (heart, kidneys, marrow, liver) For perinatal asphyxia to have been cause of later neurodevelopmental problem, must document neonatal encephalopathy

The Newborn History The baby’s history is: the family history the mother’s past medical history the mother’s pregnancy history (including any information about screening tests, amniotic fluid) the labor and delivery history (including the placenta and umbilical cord) the resuscitation history

Physical Examination Vital signs Measurements: plot on curves Gestational age assessment Overall appearance System by system

Most common anomalies noted on initial exam

Most frequent birth injuries Asphyxia Broken clavicle Facial palsy Brachial plexus injury Fractures of humerus or skull Lacerations or scalp injuries Ruptured internal organs Testicular trauma Fat necrosis

Commonest Congenital Abdominal Masses Renal (55%) Genital (15%) Gastrointestinal (15%) Liver and Biliary (5%) Retroperitoneal (5%) Adrenal (5%)

Common physical findings of clinical significance Apnea, tachypnea, grunting Bradycardia, cyanosis Hypotonia Absent or decreased femoral pulses Heart murmur Organomegaly Absent red reflex Jaundice Plethora or pallor or diffuse petechiae

Disorders of gestation length or of growth Small for gestational age: <2SD below Large for gestational age: >2SD above Prematurity: <37 weeks gestation Postmaturity: >42 weeks gestation

Small for gestational age: etiologies Constitutional: ethnicity Maternal: illness, Rx/R-OH/drugs, nutrition Placental Fetal: genetic disorder, infections (TORCH)

Small for gestational age: complications Asphyxia Meconium aspiration Congenital malformations Hypoglycemia Hypothermia Hypocalcemia Polycythemia-hyperviscosity

Small for gestational age: Management Optimal resuscitation Maintenance of body temperature Early feeds or administration of glucose Meticulous history and physical examination, including placenta Work-up for etiology

Disorders of gestation length or of growth Small for gestational age: <2SD below Large for gestational age: >2SD above Prematurity: <37 weeks gestation Postmaturity: >42 weeks gestation

Large for gestational age: Etiologies Constitutional Abnormal maternal glucose tolerance Syndromes: Beckwith-Wiedemann Sotos

Large for gestational age: Complications Asphyxia Birth trauma Hypoglycemia

Large for gestational age: Management Optimal resuscitation Early feeds or administration of glucose

Disorders of gestation length or of growth Small for gestational age: <2SD below Large for gestational age: >2SD above Prematurity: <37 weeks gestation Postmaturity: >42 weeks gestation

Risk Factors for Prematurity -previous preterm birth/labour -cervical/placental anomalies -chorioamnionitis -uterine distention -twins/multiple pregnancy -maternal medical conditions -low pre-pregnancy weight -maternal age

Risk Factors for Prematurity cigarette smoking high perceived stress bacterial vaginoses cocaine use urinary tract infection asymptomatic bacteriuria

Prematurity: Complications Respiratory distress syndrome Bronchopulmonary dysplasia Apnea of prematurity Patent ductus arteriosus Intraventricular hemorrhage Periventricular leukomalacia Necrotizing enterocolitis Sepsis Anemia Retinopathy of prematurity

Respiratory Distress Syndrome Etiology Anatomic immaturity of the lung Increased interstitial and alveolar lung fluid Surfactant deficiency Management Prevention: antenatal steroids Oxygen Positive pressure Surfactant

Courtesy of Professor Louis De Vos 17 Weeks Courtesy of Professor Louis De Vos http://www.ulb.ac.be/sciences/biodic/index.html

Courtesy of Professor Louis De Vos 22 Weeks Courtesy of Professor Louis De Vos http://www.ulb.ac.be/sciences/biodic/index.html

Courtesy of Professor Louis De Vos 25 Weeks Courtesy of Professor Louis De Vos http://www.ulb.ac.be/sciences/biodic/index.html

Bronchopulmonary Dysplasia Respiratory symptoms, oxygen requirement for at least 28 days, and X-ray abnormalities at 36 wks postconceptional age Pathophysiology: disturbed alveolarization -Lung inflammation -Mucociliary dysfunction -Airway narrowing -Hypertrophied airway smooth muscle -Alveolar collapse -Constriction of pulmonary vascular bed

Bronchopulmonary Dysplasia Management: Prevention Nutrition Oxygen +/- ventilation Bronchodilators Diuretics Steroids: inhaled vs systemic

Apnea of Prematurity Central, obstructive, or mixed Majority of <32 weeks Treat with Adequate positioning Oxygen Methylxanthines CPAP Ventilation if necessary

Patent ductus arteriosus Up to 42% of < 1500 g babies Management strategies: -preload/afterload reduction -Adequate oxygenation -Optimize pH -indomethacin -surgery -conservative management

Metabolic Problems of Prematurity Hypoglycemia Fluid/electrolyte imbalance Hypocalcemia/hypomagnesemia Hyperbilirubinemia Hypothermia

Intraventricular hemorrhage Common in < 1500 gm babies Usually evident in 1st week of life Reasons: highly vascularized germinal matrix less basement membrane to capillaries abnormal autoregulation Prognosis good for small amount bleeding in ventricles but poorer if large amount intraparenchymally or if posthemorrhagic hydrocephalus

Periventricular leukomalacia Ischemic lesion to watershed area around ventricles in premature infants Link to inflammation? Most often shows up 3-4 wks after delivery Correlated with cerebral palsy

Necrotizing Enterocolitis 1-5% NICU admissions Multifactorial etiology feeds, prematurity, ischemia, infection Diagnosis: clinical and radiologic Treatment: Decompression (NPO, NG tube) antibiotics surgery if necessary

30 week infant girl; 2 weeks old; treated for PDA with indomethacin

Triplet 2, 30 weeks; unwell on day 8 with spells, abdominal distension

Sepsis Suboptimal immune function in preemies plus poor skin barrier, indwelling catheters GBS and coliforms cause early onset sepsis < 5-7 days of life Nosocomial sepsis common in prems with most common organism = coagulase negative staphylococcus; fungi can also be problematic in > 1 week of life

Anemia of Prematurity Reasons: decreased hemoglobin at delivery decreased RBC survival blunted erythropoietin response IATROGENIC Treatment: prevention iron supplementation transfusion EPO

Retinopathy of Prematurity 40-70% NICU survivors < 1000 g Etiology: vasoconstriction leading to abnormal vascular proliferation Diagnosis: screening Treatment: close monitoring, laser if necessary

Disorders of gestation length or of growth Small for gestational age: <2SD below Large for gestational age: >2SD above Prematurity: <37 weeks gestation Postmaturity: >42 weeks gestation

Postmaturity Labour tends to be induced to avoid problems of postmaturity, however if dates not accurate may still occur Possible complications growth disturbances asphyxia meconium aspiration syndrome

Problems of the Term Newborn Respiratory Cardiac Sepsis Digestive Jaundice Anemia, polycythemia, hemorrhage Renal Endocrine Neurologic

Respiratory Distress in the Newborn Respiratory system Cardiac Infectious Neurologic Metabolic Upper airway Maternal Rx Musculoskeletal

Respiratory Problems in the Term Newborn Transient tachypnea of the newborn Pneumonia Meconium aspiration Pulmonary air leaks Congenital malformations Persistent pulmonary hypertension Pulmonary hemorrhage

Transient Tachypnea of the Newborn Failure to clear lung fluid Associated with absent or short labour or initial weak or absent respirations Improves with time

Pneumonia Can initially be difficult to distinguish from TTN/RDS Group B Strep #1 Consolidation may appear after a few days

Meconium Aspiration Syndrome Meconium-stained amniotic fluid Intrauterine insult may lead to gasping Meconium aspirated Pneumonitis Airway occlusion Pulmonary air leak syndrome May lead to persistent pulmonary hypertension

Congenital Malformations Anomalies anywhere along airways, extrinsic or intrinsic Atresias Cysts Diaphragmatic hernia

Persistent Pulmonary Hypertension Associated with asphyxia meconium aspiration sepsis Right to left shunting, persistent fetal circulation Treatment: oxygenation, ventilation maintain blood pressure pulmonary vasodilators

Congenital Heart Disease: presentations Cyanosis presents early defects with right to left shunts TOF, tricuspic atresia, TGA, TAPVR, truncus arteriosus, pulm. atresia Congestive heart failure fewer compensatory mechanisms so common and can occur very quickly tachycardia, tachypnea, hepatomegaly, feeding difficulty, cardiomegaly, diaphoresis

Presentations of Congenital Heart Disease Murmurs Dysrhythmias

Congenital heart disease: Most commonly diagnosed Ventricular Septal Defect Transposition of the Great Vessels Tetralogy of Fallot Coarctation of the Aorta Patent Ductus Arteriosus Endocardial Cushion Defect Hypoplastic Left Heart

Sepsis: risk factors Preterm rupture of membranes Prolonged rupture of membranes Maternal group B strep carriage Chorioamnionitis

Neonatal Sepsis THINK OF IT! Signs may be subtle, non-specific Incidence bacterial sepsis = 1-5/1000 live births Commonest organisms: group B streptococcus gram negatives (E coli, Klebsiella) enterococcus, H flu, staph species listeria Work up and treat if suspect sepsis Use broad spectrum antibiotics

Ophthalmia neonatorum 1st days - differentiate chemical vs infected 2nd-3rd wk - viral or bacterial Gonococcal: within 5 days of birth gram negative intracellular diplococci if suspect, Penicillin asap highly contagious Chlamydia: 5-14 days conjunctival scraping topical antibiotics

Congenital Infections CMV: 5-25/1,000 live births asymptomatic vs severe symptoms microcephaly, thrombocytopenia, hepatosplenomegaly, chorioretinitis sequelae of hearing loss and developmental delay Rubella 0.5/1,000 cataracts, rash, congenital heart disease, developmental delay

Congenital Infections Toxoplasmosis: 0.5-1.0/1,000 hydrocephalus, cranial calcifications, chorioretinitis Syphilis: 0.1/1,000 snuffles, osteochondritis/periostitis, rash Herpes: vesicles, keratoconjuntivitis, CNS findings

Congenital syphilis Treat mother no matter what stage of pregnancy If adequate maternal treatment and no signs of infection in newborn, give one dose IM penicillin If inadequate maternal treatment, give 10 days of IV penicillin

Neonatal herpes simplex Only about 1/3 mothers have overt signs Infection can be disseminated or local Usually present at 5-10 days of age If suspect: Cultures, PCR Treat with acylovir

Maternal hepatitis B carrier Give baby hepatitis vaccine as soon as possible after birth (first 12 hours) Bath Universal precautions Immune globulin in first 7 days

HIV Virus can be transmitted transplacentally, intrapartum, or postpartum Screen mothers Treat mothers with antiretrovirals Treat babies with AZT for 6 wks Universal precautions Look for other infections (HepB, HepC)

Digestive Disorders Vomiting Diarrhea Constipation

Vomiting in the Newborn Not uncommon for some vomiting in 1st few hours after birth Overfeeding, poor burping DDx: Gastrointestinal obstruction Increased intracranial pressure Bilious vomiting is a medical emergency!

Upper G-I problems causing vomiting Esophageal: first feed, soon after feed excessive drooling if T-E fistula, risk aspiration Small bowel atresias Malrotation and volvulus Achalasia Chalasia/GER Pyloric stenosis }Need to r/o

Non-GI causes of vomiting Sepsis Adrenal hyperplasia Meningitis UTI Milk allergy

Lower GI Obstruction Initially, distention, failure to pass meconium… vomiting is later sign Extrinsic vs intrinsic obstruction DDx: Imperforate anus, Hirschprung, meconium ileus, meconium plugs, ileal atresia, colonic atresia

Colonic atresia

Hirshsprung

Distal ileal volvulus

Constipation > 90% pass meconium in first 24 h Present at birth, consider causes of GI obstruction Present after birth, consider Hirschprung, hypothyroidism, anal stenosis NB some breastfed babies normally stool only once every 5-7 days

Diarrhea Infection E coli, salmonella, echovirus, rotavirus, adenovirus Watch for fluid and electrolyte imbalance

Jaundice First 24 h, always abnormal Etiology: unconjugated 1. RBC destruction/hemolytic : isoimmune, RBC membrane, enzymes, hemoglobinopathies Hematoma Sepsis (mixed hemolytic and hepatocellular damage Hypoxia 2. Congenital/metabolic: Criggler-Najar Hypothyroidism, galactosemia

Jaundice Later onset: conjugated Hepatocellular damage: Viral bacterial Metabolic: CF, tyrosinemia 2. Post hepatic: biliary atresia choledochal cyst

Jaundice - Work-Up History and physical examination Bilirubin - total and direct Blood type and Coomb’s Hemoglobin Reticulocyte count Smear Septic workup

Risk factors for kernicterus Prematurity Hemolysis Asphyxia Acidosis Infection Cold stress Hypoglycemia

Treatment of Jaundice Nutrition/hydration Phototherapy Exchange transfusion

Anemia Hemorrhage feto-maternal feto-placental feto-fetal intracranial or extracranial rupture of internal organs Hemolysis Treatment: Transfuse if necessary

Polycythemia-Hyperviscosity Syndrome Hematocrit > 65 or 70% “Sludging” of blood in organ May present with: respiratory symptoms CNS symptoms thrombocytopenia Treat by partial exchange transfusion

Bleeding in the Newborn Hemorrhagic disease of the newborn Thrombocytopenia immune infection related congenital Disseminated intravascular coagulation

Renal issues in the Newborn Most common site of congenital malformations and hence abdominal masses Renal vein thrombosis: complication of infant of diabetic mother or polycythemia Increased risk of UTI’s in uncircumcised males (but still not as high as infant females) All newborns have poor concentrating ability; small prematures at high risk for fluid/electrolyte imbalance

Endocrine issues in the Newborn Congenital hypothyroidism Screen because too late if wait Signs = poor feeding, constipation, prolonged jaundice, large fontanelles, umbilical hernia, dry skin

Endocrine Issues in the Newborn Congenital adrenal hyperplasia 21-hydroxylase deficiency most common Signs = vomiting, diarrhea, dehydration, shock, convulsions, clitoris or phallic enlargement Watch for electrolyte imbalance If suspect, send lab tests and treat

Endocrine issues in the Newborn Infant of diabetic mother Congenital malformations (especially important to have good control preconception) Growth disturbances Metabolic disturbances: glucose, Ca+ + Respiratory distress syndrome and transient tachypnea of the newborn: more prone Polycythemia: jaundice Cardiovascular problems: hypertrophic cardiomyopathy

Hypoglycemia BS <2.6 prem and bottle fed term BS <2.0 breastfed ** No clear safe cutoff for all Lack of supply Lack of reserve (low glycogen): IUGR Inability to use/produce: metabolic Increased utilization: sepsis Increased insulin production

Hypoglycemia Treat: supply 4-6 mg/kg/min term 6-8 mg/kg/min prem Look for cause, especially if severe or persists beyond 48-72h of life

Neonatal seizures: etiology

The Hypotonic Infant: Etiologies Central nervous system disease Spinal cord diseases Diseases of the peripheral nerve Diseases of the neuromuscular junction Muscle Diseases Systemic diseases Metabolic diseases

Work-up of Hypotonic Infant Exhaustive history Complete physical examination Imaging: CXR, U/S, CT, MRI Nerve conduction velocity, electromyography Serum CPK, AST, CSF protein Muscle biopsy, nerve biopsy Molecular genetics (myotonic dystrophy, Prader-Willi) Other