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

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

3. Infant mortality: 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

4. Gestational age and size GestationSize 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

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

7. Fluid filled alveoli in utero Diminished blood flow through fetal lungs

8. Importance of first breath

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

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

14. The Apgar Score

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

16. n Prophylaxis for common problems –Eye care: erythromycin ointment –Vitamin K: 1 mg IM n 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) n Blood group and Coombs if mother rH neg

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

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

19. The Newborn History n 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

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

21. Most common anomalies noted on initial exam

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

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

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

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

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

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

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

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

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

51. Large for gestational age: Complications n Asphyxia n Birth trauma n Hypoglycemia

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

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

55. 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

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

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

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

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

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

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

67. Bronchopulmonary Dysplasia n 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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

98. n Murmurs n Dysrhythmias Presentations of Congenital Heart Disease

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

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

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

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

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

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

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

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

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

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

114. Digestive Disorders n Vomiting n Diarrhea n Constipation

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

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

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

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

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

127. Diarrhea n Infection –E coli, salmonella, echovirus, rotavirus, adenovirus n Watch for fluid and electrolyte imbalance

128. Jaundice n First 24 h, always abnormal n 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

129. n n Later onset: conjugated 1.Hepatocellular damage: ViralViral bacterialbacterial Metabolic: CF, tyrosinemiaMetabolic: CF, tyrosinemia 2. Post hepatic: biliary atresiabiliary atresia choledochal cystcholedochal cyst Jaundice

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

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

132. Treatment of Jaundice n Nutrition/hydration n Phototherapy n Exchange transfusion

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

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

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

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

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

139. Endocrine Issues in the Newborn n 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

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

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

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

145. Neonatal seizures: etiology

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

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