Prematurity

 Term is 37-42 weeks

 Birth <37 weeks gestation
 1/3 of all infant deaths
 ↑mortality with ↓gestation
 Short-term
o eg, respiratory and cardiovascular complications
 Lon-term
o eg, neurodevelopmental disabilities such as cerebral palsy
 Obliged to resus > 26 weeks, given option to resus with counselling beforehand
 Definitions
o Late preterm birth — GA between 34 and less than 37 weeks
o Very preterm birth — GA less than 32 weeks
o Extremely preterm birth — GA at or below 28 weeks
o By birth wieght
 Low birth weight (LBW) — BW less than 2500 g
 Very low birth weight (VLBW) — BW less than 1500 g
 Extremely low birth weight (ELBW) — BW less than 1000 g
 Complicationof VLBWs:
o Respiratory distress: 93 percent
o Retinopathy of prematurity: 59 percent
o Patent ductus arteriosus: 46 percent
o Bronchopulmonary dysplasia: 42 percent
o Late-onset sepsis: 36 percent
o Necrotizing enterocolitis: 11 percent
o Grade III intraventricular hemorrhage (IVH) and Grade IV IVH: 7 and 9 percent
o Periventricular leukomalacia: 3 percent
 Complications of late pre-term
o Hyperbilirubinemia: 59 percent
o Acute respiratory disease: 28 percent
o Hypoglycemia: 16 percent
o Bacterial infection: 15 percent

Complications
Hypothermia
 Rapid heat loss occurs in premature infants because of their relatively large body surface area and inability
to produce enough heat. Heat is lost by conduction, convection, radiation, and evaporation.
 may contribute to metabolic disorders such as hypoglycemia or acidosis
 admission temperature inversely related to mortality and late-onset sepsis
 Management in delivery room:
o Maintaining the delivery room temperature at a minimum of 26ºC
o Drying the baby thoroughly immediately after birth
o Removal of any wet blankets
o Use of prewarmed radiant heaters if resuscitation is necessary
 Once in the NICU, premature infants should be cared for in an incubator or radiant warmer to avoid
hypothermia

Respiratory abnormalities
 RDS
 Bronchopulmonary dysplasia, also known as chronic lung disease, is a late respiratory complication that
commonly occurs in VLBW infants
o defined as oxygen dependency at 36 weeks postmenstrual age
 Apnea of prematurity
Cardiovascular abnormalities
 Patent ductus arteriosus
o Symptomatic PDA is common in premature neonates
o Normally →
 ↑O2 when starting breathing → ↓ prostaglandins leads to DA fibrosis and contraction →
ligamentum arteriousm
 Ibuprofen also blocks prostaglandins
o Ibuprofen also however, leads to ↓ blood flow to kidneys → RENAL FAILURE
 Monitor renal output
 In normal newborns, the DA is substantially closed within 12–24 hours after birth,
and is completely sealed after 3 weeks
 Foramen ovale closes due to ↑ LA pressure
 First breath → ↑pulmonary vessel diltation as oxygen is most potent vasodilator
o PDA shunts blood flow from left-to-right resulting in increased flow through the pulmonary
circulation and decreased perfusion of the systemic circulation
 Pulmonary odema, hypoxia and metabolic acidosis
o Significant shunting may present with a variety of symptoms including apnea, respiratory distress, or
heart failure
 Systolic murmur
o Treatment
 Ligation, ibuprofen
o MEASURE ALL NEONATAL OXYGEN SATURATION ON RIGHT HAND
 Pre-ductal circulation (same as brain)
 Blood pressure
o low blood pressure (BP), without evidence of shock, is commonly observed in neonates
o management:
 Volume expansion — Both crystalloid (eg, normal saline) and colloid (eg, fresh frozen plasma
and albumin) solutions
 Inotropic therapy
 Dopamine infusion
 Systemic glucocorticoid therapy

Intraventricular hemorrhage
 usually occurs in the fragile germinal matrix and increases in frequency with decreasing birth weight
 ↑risk of bleeding due to immediate change in vascular pressures
o Ultrasound day 1, 2, 7 through anterior fontanelle
o Ultrasound at 1 month for PVS
 Periventricular leukomalacia
 Scarring of white matter around ventricles
 Sign of hypoxia
 ↑risk CP
Glucose abnormalities
 Disorders in glucose supply or metabolism can result in hypoglycemia or hyperglycemia

Necrotizing enterocolitis
 2 to 10 percent of VLBW infants
 Survivors are at increased risk for growth delay and neurodevelopmental disabilities
 10% of preterm infants with NEC will have long-term gastrointestinal difficulties with persistent loose stools
or frequent bowel movements.

Infection
 Late-onset sepsis
o >3 days, common in premature
 Mainly gram-positive, but also fungal infections
 increased likelihood of poor neurodevelopmental outcome and growth impairment.
Retinopathy of prematurity
 developmental vascular proliferative disorder that occurs in the incompletely vascularized retina of
premature infants
 Due to sudden change in vascular perfusion → ↑O2 → vascular growth in retina → scarring and shrinking →
retinal detatchment
 begins about 34 weeks postmenstrual age
 should be monitored fortnightly
 babies usually adapt in mild cases

Feed volumes
o 60mL first day
o +20mL every day after, until 150mL for term, higher for pre-term

Explain in detail the pathophysiological basis of common conditions in the neonatal

period
INFANT MORTALITY
 9-10/1,000 births
 causes
• congenital
• prematurity (RDS, intracranial haemorrhage)
• asphyxia
• infections
• sudden infant death syndrome
NORMAL BABY AT TERM
 HR 120-160/per min
 RR 40-60/per min
 weight 2500-4500 g
 glucose > 2.2
 BP systolic 50-80, diastolic 30-40 (dependent on GA)
Routine care
 Normally should breath spontaneously

The first minutes

 Establishing breathing
o Stimulated by cooling of the face and physical stimuli and hypoxia and acidosis
o Usually within seconds of birth
 Heat loss after birth
o Evaporative cooling occurs very quickly after birth
o Dry and wrap the baby
o Rectal temperature stabilises at around 37 °C by 1hr
o Warming under incubator
 Umbilical cord
o Clamped and cut cleanly close to skin
o Antiseptic solution, such as chlorhexidine in alcohol, may be applied daily until the cord stump drops
off
 First hours
o washed with soap and water
o Often the baby will be very alert and breast-feeds should be started at these times.
 Vitamin K
o All babies receive vitamin K mixed micelles (Konakion) 0.1 mL i.m
o eradicates haemorrhagic disease of the newborn
o Gut flora not yet developed enough to produce Vit K
 Vit K needed for activation of factors 2,7,9,10 in coagulation cascade
 First day
o Suck and swallow easily: if s/he does not, consider unrecognised prematurity, a congenital
abnormality, hypoglycaemia or infection.
o Pass urine: many babies pass urine at birth and it is missed. If a boy does not pass urine in the first
24 h, consider posterior urethral valves. This requires careful urological investigation. A reasonable
screening test is to ultrasound the bladder and see if it empties completely with micturition.
o Pass meconium within the first 48 h: if not, consider bowel obstruction or Hirschsprung disease.
 First examination
o detect congenital abnormalities
o Observation: before disturbing the baby observe the posture, behaviour, general appearance, colour
and well-being.
o Chest: while the baby is quiet examine the heart sounds, rate, presence of femoral pulses and the
pulse characteristics. Many babies have a very soft murmur in the first few days. Recession and
laboured breathing are the most important signs of respiratory distress.
o Head and neck: look for scalp defects; fractures; haematomas; lacerations; eye size, anatomy and
red reflex; neck cysts, lumps or fistulae; cleft palate; tongue size and shape; ear position, shape, size
and tags or fistulae and facial symmetry when crying. A cephalohaematoma is a soft boggy swelling
over one bone (usually the parietal) due to blood under the periosteum. It needs no treatment.
Beware of a generalised boggy swelling all over the scalp in a shocked baby. It may be a subgaleal
haemorrhage. Babies can bleed profusely into these and they are a neonatal emergency.
o Abdomen: feel for masses (liver, spleen, kidneys, bladder and ovaries), distension and tenderness.
Examine the genitalia, anus and inguinal and umbilical region for herniae. The umbilicus should be
clean and dry. The liver is often just palpable or percussible in normal babies.
o Limbs: examine for abnormal fingers, hands, toes and feet; posture of the hands and feet; and the
flexibility of the joints.
o Hips: carefully examine for congenital dislocation by observation and specific palpation
o Measurement: naked weight, length and head circumference should be recorded and plotted on the
growth chart in the baby’s record book.
APGAR scores
o Taken at 1, 5 and sometimes 10 minutes
o ↑morbidity of < 7 at 5 minutes
ROUTINE NEONATAL CARE
 eye care - erythromycin ointment to prevent ophthalmia neonatorum -
 gonorrhea, chlamydia
 vitamin K - to avoid haemorrhagic disease of newborn
 HBIG plus vaccine if mother is Hep B +ve
 screening test
• in all neonates: PKU, TSH usually after 24 hours of life
• if indicated: blood group, sickle cell, G6PD deficiency (varies by province)
• blood group and direct antiglobulin test if mother Rh-ve

COMMON PROBLEMS
Neonatal Jaundice
 very common - 65% of newborns
 85 umol/l total serum bilirubin (TSB) in blood to
be visible
 look at sclera, mucous membranes, palm creases
 Almost always due to unconjugated bilirubin
Risk Factors
 prematurity
 acidosis
 sepsis
 hypoalbuminemia
 dehydration

Bilirubin
 Protects newborn from oxygen toxicity in the first
days of life
 is produced by the breakdown of heme (iron
protoporphyrin) in the reticuloendothelial system
and bone marrow
o Heme is cleaved by heme oxygenase to
iron, which is conserved; carbon
monoxide, which is exhaled; and biliverdin, which is converted to bilirubin by bilirubin reductase
o unconjugated bilirubin is bound to albumin and carried to the liver, where it is taken up by hepatocytes
o in the precence of enzyme UDPGT, bilirubin is conjugated to one or two glucuronide molecules
o Conjugated bilirubin is then excreted through the bile to the intestine
o In the presence of normal gut flora, conjugated bilirubin is metabolized to stercobilins and excreted in
the stool
 Absence of gut flora and slow GI motility, both characteristic of the newborn, cause stasis of
conjugated bilirubin in the intestinal lumen, where mucosal -glucuronidase removes the
glucuronide molecules and leaves unconjugated bilirubin to be reabsorbed (enterohepatic
circulation)
 Low ability to bind to albumin → ↑free bilirubin when drugs, sepsis or acidosis is present
Causes:
< 24 Hours of Age
 always pathologic and requires investigation
o blood group, baby’s serum bilirubin (total and
unconjugated), direct Coombs test,
haemoglobin, white cell count, platelets and
peripheral smear, LFTs
o also test mothers red cell antibodies
 admit to special care nursery
 Mostly caused by haemolysis
o Rh or ABO incompatibility between mother and fetus
o Severe haemolysis → rapid ride in serum bilirubin in over a few hours
o internal hemorrhage
 sepsis/congenital infection: TORCH
 Further investigate if no haemolysis, or conjugated hyperbilirubinaemia present
 Phototherapy should be commenced if the bilirubin level is >150 μmol/L in the first 24h in a term infant
 Immediate exchange transfusion if due to rhesus incompatibility and infant anaemia ( Hb <110 g/L)
 Frequent monitoring of bilirubin levels is essential as rapid changes may occur

> 24 Hours of Age

 Criteria for physiological:
o The jaundice appeared on day 2–4.
o The baby is not premature.
o The baby is well (afebrile, feeding well and alert).
o The baby is passing normal-coloured stools and urine.
o There are no other abnormalities.
o Bilirubin levels are not above treatment threshold.
 In 1/3 of term babies, usually visually jaundiced by 2-4 days
 Visible only once serum bilirubin is >85–120 μmol/L
o But rarely exceeds 220 μmol/L in physiological
jaundice
 If uncongonjugated >220 μmol/L consider other causes (such as infections)
 Physiologic causes:
o immature liver enzymes, increased hematocrit with decreased RBC lifespan overload the liver,
absence of intestinal flora, slow intestinal motility, and increased enterohepatic circulation of
bilirubin in the first days of life
o onset day 2-5 in full-term, 6-7 in preterm infants, usually
o peaks 2 days after onset
 if not physiologic, then investigate: blood group, Coombs, haemoglobin, peripheral smear
o maternal antibodies (Coombs test–positive)
  ABO blood group incompatibility in type O mother, mild haemolysis
 Rh-isoimmunisation
 Affected neonates are often anemic at birth, and hemolysis rapidly causes
hyperbilirubinemia and more severe anemia
 erythroblastosis fetalis, is characterized by life-threatening anemia, generalized
edema, and fetal or neonatal heart failure
o transfusion of the fetus with Rh-negative cells and phototherapy with IVIG
o abnormal red cell membranes (spherocytosis)
 hemolysis by decreasing red cell deformability
 splenomegaly may be present
o consider septic workup CBC, diff, C&S urine and blood, ± CSF, ± chest x-ray
o Pathological causes:
 increased haemolysis
o G6PD deficiency → most common red cell enzyme defect causing hemolysis,
especially in infants of African, Mediterranean, or Asian descent
 bruising, haemorrhage, hematoma, cephalohematoma
 polycythemia
 drugs
 sepsis/congential infection: TORCH
 dehydration
 Well, term infants with no haemolysis are at minimal risk of kernicterus. Infants who are at higher risk of
kernicterus are:
o Unwell infants (particularly those exposed to hypoxic insults).
o Infants with haemolysis.
o Preterm infants.
 These infants should have treatment started at lower bilirubin levels.

Gilbert Syndrome
 This is a common mild autosomal dominant disorder characterized by decreased hepatic UDPGT activity
 Affected individuals tend to develop hyperbilirubinemia in the presence of conditions that increase bilirubin
load, including G6PD deficiency.
 They are also more likely to have prolonged neonatal jaundice and breast-milk jaundice.

Prematurity
 Premature infants often have poor enteral intake, delayed stooling, and increased enterohepatic circulation, as
well as a shorter red cell life.
 Infants at 35–36 weeks' gestation are 13 times more likely than term infants to be readmitted for
hyperbilirubinemia.
 Even early-term infants (37 weeks' gestation) are four times more likely than term neonates to have TSB greater
than 224 mmol/L.

Prolonged Neonatal Jaundice (> 1 Week of Age)

 Usually secondary to breastfeeding and is benign
o 10% breast fed infants
o inhibition of glucuronyl transferase activity
o may persist up to 4-6 weeks, no treatment needed, continue with breastfeeding
o not associated with kernicterus
o Rule out other causes first
 Check serum bilirubin to determine conjugated or unconjugated
o Unconjugated:
• Hypothyroidism
• infection
• red cell enzyme abnormalities
 causes haemolysis
 Most likely G6PD deficiency
 • ADMIT to hospital immediately
o Conjugated(>25% total or >25 μmol/L)
• Uncommon, always pathological
• impaired excretion (e.g. biliary atresia, choledochal cyst)
 Acholic pale grey stools, dark urine
 failure to thrive, malabsorption
• neonatal hepatitis
• inborn error of metabolism (e.g. galactosemia)
• parenteral nutrition
 Other causes:
o conjugation dysfunction (e.g. Gilbert's disease, Crigler-Najjar Syndrome)

Bilirubin Toxicity
 Unconjugated bilirubin anion is the agent of bilirubin neurotoxicity.
 The anion binds to the phospholipids (gangliosides) of neuronal plasma membranes causing injury, which then
allows more anion to enter the neuron.
 Intracellular bilirubin anion binds to the membrane phospholipids of subcellular organelles, causing impaired
energy metabolism and cell death.
 Risk depends on the duration of hyperbilirubinemia, the concentration of serum albumin, associated illness,
acidosis, and the concentrations of competing anions such as sulfisoxazole and ceftriaxone
 Premature infants are at greater risk than term infants because of the greater frequency of associated illness
affecting the integrity of the blood-brain barrier, reduced albumin levels, and decreased affinity of albumin-
binding sites

Acute Bilirubin Encephalopathy

 Lethargy, poor feeding.
 Irritability, high-pitched cry.
 Arching of the neck (retrocollis) and trunk (opisthotonos).
 Apnea, seizures, coma (late).

Chronic Bilirubin Encephalopathy (Kernicterus)

 CNS toxicity (associated with increased unconjugated bilirubin + saturation of albumin or open blood brain
barrier, basal ganglia targeted)
 irreversible brain injury
 Symptoms
o Extrapyramidal movement disorder (choreoathetoid cerebral palsy).
o Gaze abnormality, especially limitation of upward gaze.
o Auditory disturbances (deafness, failed auditory brainstem evoked response with normal evoked
oto-acoustic emissions, auditory neuropathy, auditory dyssynchrony).
o Dysplasia of the enamel of the deciduous teeth.
 >340 μmol/L bilirubin
 MRI is diagnostic

Treatment
 maintain good hydration and normal acid-base status
 1st line therapy: phototherapy - photoisomerization (blue light most effective)
 exchange transfusion, depending on level of bilirubin, age, weight
 treat any underlying cause
 do not interrupt breastfeeding in healthy term newborns

Phototherapy
 most common treatment for indirect hyperbilirubinemia
 Light of wavelength 425–475 nm (blue-green spectrum) is absorbed by unconjugated bilirubin in the skin
converting it to a water-soluble stereoisomer that can be excreted in bile without conjugation
 Cover infants eyes
 Started electively when TSB > 102 mmol/L
Exchange Transfusion
 extreme indirect hyperbilirubinemia
 admit to NICU
 Albumin (1 g/kg) will aid in binding and removal of bilirubin
 Double-volume exchange transfusion (approximately 160–200 mL/kg body weight
 invasive, risky, and infrequently performed
o Mortality is 1–5%
o 5–10% risk of serious complications such as necrotizing enterocolitis (NEC), infection, electrolyte
disturbances, or thrombocytopenia

RESPIRATORY DISTRESS IN THE NEWBORN

Presentation
 tachypnea > 60 / per min
 audible expiratory grunting
o Due to closing of glottis to maintain positive pressure on early expiration → PEEP
 Increased work of breathing (recession of the lower chest wall and upper abdomen).
o intercostal retractions/indrawing
o Due to negative pressure and softer bones
o Children mainly breath with diaphragm, underdeveloped accessory muscles
 nasal flaring
o ↑lumen diameter → ↑flow
 duskiness/central cyanosis
 decreased A/E on auscultation
 tachycardia > 160 / per min
 SEND TO NICU if needing >40% O2 to maintain >88% saturation
Diagnosis
 chest x-ray
 ABG, CBC, blood glucose
 blood cultures, Gram stain
Differential Diagnosis
 pulmonary
o respiratory distress syndrome (RDS)
o transient tachypnea of the newborn (TTN)
o meconium aspiration (group B strep and others)
o atelectasis
o pleural effusions
o pneumothorax
o congenital lung malformations
 cardiac
o congenital heart disease (cyanotic, obstructive, LR shunt)
o persistent pulmonary hypertension (PPHN)
 hematologic  metabolic
o blood loss o hypoglycemia
o polycythemia o inborn errors of metabolism
 infectious  neuromuscular
 anatomic o CNS damage (trauma, hemorrhage)
o tracheoesophageal fistula o medication (maternal sedation)
o congenital diaphragmatic hernia o anomalies (e.g. Werdnig-Hoffmann
disease)
o drug withdrawal syndromes

Respiratory distress syndrome (RDS) or hyaline membrane disease

 Primarily caused by immaturity (usually <30 weeks gestation)- most common cause of respiratory death in pre-
term infants
o Incidence ↑with ↓ gestation
o Can occur in term babies, especially if CS delivery
 Due to immaturity of surfactant lung structure and fluid clearance→ poor lung compliance due to high alveolar
surface tension and atelectasis → damages epithelium and protein exudes on to the surface → forming hyaline
membranes → hypoxia + acidosis
o Respiratory distress (oxygen requirement, work of breathing) typically increases over 12–24 h before
improvement is seen
o Rapid deterioration may occur leading to respiratory failure (rising arterial CO2 level).
 Risk factors:
o premature babies (5% risk @ 33 weeks, 65% risk @ 29 weeks)
o infants of diabetic mothers (insulin inhibits the cortisol surge necessary for surfactant synthesis)
o CS (reduced with antenatal steroids to mother)
o asphyxia, acidosis
o second of twins
o males: females = 2:1
 Diagnosis: clinical features + CXR [fine reticulogranular (‘ground glass’) appearance with air bronchograms]
 Mx : Respiratory support with oxygen, assisted ventilation (CPAP, IPPV) and surfactant therapy (bovine or
synthetic)
o Surfactant therapy through ET tube for all under 28 weeks
 Prevention
 minimize prematurity
 monitor L/S ratio
 steroid therapy (Celestone) for mothers 24 hours prior to delivery
 of premature infants
 Prognosis
o self-limited disease, tends to improve after 72 hours without complications
o in severe prematurity and/or prolonged ventilation, increased risk of bronchopulmonary dysplasia
 Complications
o PDA
o bronchopulmonary dysplasia
o retinopathy of prematurity
o pulmonary air leaks (pneumothorax)
o intracerebral/intraventricular haemorrhage

Transient tachypnoea of the newborn or ‘wet lung syndrome’ TTN

 delayed clearance of fetal lung fluid → accumulation of fluid in peribronchial Iymphatics and vascular spaces →
tachypnea
 Commonly occurs in babies born near term by elective Caesarean section
 Also known as persistent postnatal pulmonary edema or RDS type 2
 Clinically:
o tachypnoea within the first few hours of life respiratory distress
o mild retractions, grunting, without signs of severe
 Presents as mild respiratory distress and lasts for up to 1–2 days.
 Diagnosis by CXR
o Coarse streaking of lung fields with fluid in the fissures.
 Treatment
o supportive: O2, fluids, nutrition
o The baby is usually not very ill, needing <30% oxygen

Pneumonia
 Group B β-haemolytic streptococcus (GBS) is the most common and serious cause of pneumonia and
septicaemia in the newborn
o Acquired at birth from a colonised mother
 o Infections usually ascend from the genital tract before or during labor, with the vaginal or rectal flora the
most likely agents
o Therefore consider in infants with prolonged rupture of membranes, chorioamnionitis, or maternal fever
 Other organisms such as E.Coli also
 presents early with severe and progressive respiratory failure → rapid progression to collapse and death
 Later infections → lethargy, temperature instability, poor feeding, respiratory difficulty, apnoea or poor
perfusion
 CXR similar to severe RDS, not diagnostic
o Temperature unstable, ↑WBC, neutropenia, ↑CRP
 Treat all with broad-spectrum antibiotic penicillin and gentamicin until cause is confirmed

Meconium aspiration syndrome (MAS)

 Hypoxia before delivery may cause the baby to gasp in utero and inhale meconium → respiratory difficulty
 10-15% of all births are meconium stained, ~5% of meconium stained infants get MAS
 higher incidence with thick meconium
 respiratory distress within hours of birth - tachypnea, hypercarbia, small airway obstruction, chemical
pneumonitis
 Associated with pulmonary hypertension and right-to-left shunting
 complications: hypoxemia, acidosis, PPHN, 11% pneumothorax, 30% mechanical ventilation, 4% mortality
 CXR shows hyperinflation and patchy consolidation(pneumothorax and pneumomediastinum may follow) or a
diffuse hazy appearance
 Management
o Oxygen mainstay, CPAP or ventilation may be required
o Antibiotics to counter infections
o May benefit from surfactant replacement
 Prevention
o careful in utero monitoring, suction naso/oropharynx at perineum, then intubate and suction below
cords at birth

Pneumothorax
 May occur spontaneously at birth, or secondary to other lung disease
 1% of all deliveries
 Risk is increased by manipulations such as positive-pressure ventilation (PPV) in delivery room
 Respiratory distress (primarily tachypnoea) is present from birth and typically is not severe
 Diagnosis by CXR
 main treatment is an intercostal catheter with an underwater drain at low negative pressure
o Asymptomatic pneumothorax usually needs no treatment.
o supplemental O2 and watchful waiting
 ↑risk of renal abnormalities → consider renal USS

Tension pneumothorax
• This is a medical emergency. There may be no time for a chest radiograph
• If a baby is deteriorating rapidly despite full resuscitation this diagnosis should be considered, particularly if there is
poor air entry on auscultation to one or both sides of the chest.
• In the event of severe deterioration, aspirate the chest using a 25-gauge scalp vein needle attached via a three-way
tap to a 10 mL syringe. The needle is inserted into the second or third intercostal space in the midclavicular line.
Aspirate the chest with the 10 mL syringe. Then insert intercostal catheter

Upper airway obstruction

 Nasal
o URTI
o choanal atresia (obstruction to the back of the nose)
 suspect if babys condition improves when opening mouth
o traumatic deviated nasal septum
o presents with difficulty in breathing or feeding
  Oral
o macroglossia may be associated with Beckwith–Wiedemann syndrome or hypothyroidism.
o Micrognathia may result in a tongue that obstructs the pharynx
o Pierre Robin sequence is an association of mandibular hypoplasia (micrognathia), cleft palate and
upper airways obstruction caused by the tongue falling back into the cleft palate.
o usually a soft inspiratory stridor associated with subcostal and intercostal indrawing of variable
severity
o babies often adopt an extensor posture in an attempt to relieve their obstruction
o Presentation at 2–4 weeks of age
o Insertion of a nasopharyngeal tube to relive obstruction
 Larynx: laryngomalacia, subglottic stenosis, laryngeal infl ammation or occasionally vocal cord palsy, present
with inspiratory stridor and suprasternal and lower chest indrawing.
 Trachea: tracheal obstruction presents with an inspiratory and expiratory wheeze and lower chest
indrawing. It may be due to tracheomalacia or a vascular ring.

Non-pulmonary causes of respiratory diffi culty

Cardiac
• Left-to-right shunts or left-sided obstructions cause pulmonary oedema.
Pulmonary hypertension
• Presents with an increased oxygen requirement and little respiratory difficulty. Chest radiograph often reveals clear
lung fi elds.

Management of respiratory diseases

Before birth
• If possible, anticipate high-risk babies and transfer to a hospital with a NICU for delivery and neonatal care.
• Betamethasone given to mothers with threatened preterm labour reduces the severity of hyaline membrane
disease, halves the mortality and incidence of brain haemorrhages and improves long-term outcome.
General care
• Observation, usually in an incubator.
• Temperature control is essential – aim for a rectal temperature of 37 °C (±0.3 °C) or axillary temperature range
36.8 °C (±0.3 °C).
• Measure blood glucose, electrolytes, full blood count and CRP.
• Take a blood culture (but not an LP in the acute stage).
• Avoid enteral feeds initially as they worsen respiratory distress via splinting the diaphragm.
• Give i.v. fl uids.
• Treat with benzylpenicillin and gentamicin until blood cultures are negative.

Respiratory care
• A chest radiograph provides the diagnosis in most cases of respiratory difficulty in neonates.
• Monitor cardiorespiratory and blood gas measurements.
• Arterial pH should be maintained at 7.35–7.45. A level of 7.25 (or just under) may be acceptable, depending on the
circumstances.
• Arterial Pco2 should be 40–60 mmHg.
• Arterial Po2.
A low Po2. means the baby is hypoxic. If the pH is also low, the baby may have a metabolic acidosis and a
higher level of inspired oxygen is required. If this does not improve oxygenation, suspect cyanotic heart
disease as a cause
• Arterial bicarbonate should be 22–26 mmol/L. A low level is due to a metabolic acidosis. The pH will also be low
unless the baby has compensated by blowing off CO2.
• Base excess should be between +3 and −3. If low it indicates a metabolic acidosis. A level of −5 is usually tolerated
in small babies without a signifi cant change in pH.
• In ventilated babies with RDS, especially those <30 weeks’ gestation, surfactant should be given via the
endotracheal tube as early as possible.
• The inspired oxygen requirement is initially determined using a pulse oximeter. Aim for an oxygen saturation of
88–95%.
• Arterial blood gases should be measured in babies needing >30% oxygen. Aim for a PaO2 level of 50–80 mmHg.
• Nasal continuous positive airway pressure (CPAP) is effective for treating RDS or apnoea.
• The decision to ventilate a baby is made on the following criteria:
– Apnoea not responding to simple treatment.
– Unsatisfactory arterial blood gases: pH <7.25 with a Pco2 >60 mmHg, or inspired oxygen >60% despite
treatment with CPAP (up to 10 cm H2O).

DIAPHRAGMATIC HERNIA
 Posterolateral or Anteromedial
 clinical features
o respiratory distress, cyanosis
o scaphoid abdomen
o affected side dull to percussion and breath sounds absent;
 may hear bowel sounds instead
o asymmetric chest movements, trachea deviated away from affected side
o may present outside of neonatal period
 chest x-ray: portion of GI tract in thorax (usually left side),
 displaced mediastinum
 treatment: surgical
 prognosis: 50% survival overall
o associated with a high incidence of pulmonary vascular
 anomalies, hypoplastic lungs

PERSISTENT PULMONARY HYPERTENSION (PPHN)

 R → L shunt through PDA / foramen ovale / intrapulmonary
 channels, decreased pulmonary blood flow creates hypoxemia leading to further pulmonary vasoconstriction
 risk factors: abruption / placenta previa, asphyxia, MAS, RDS,
 sepsis, structural abnormalities (Potters / diaphragmatic hernia)
 treatment: O2 given early, tapered slowly, minimize stress / hypoxia, if mechanical ventilation is
unsuccessful, extracorpreal
 membrane oxygenation (ECMO) may be required

BRONCHOPULMONARY DYSPLASIA (BPD)

 usually after prolonged intubation/ventilation with high oxygen
 concentration ( incidence with maturity)
 persistent respiratory distress
o decreased compliance, increased resistance, pulmonary edema
o hypoxemia, hypercapnia, may have apnea and bradycardia
 may have cardiac component (congestive heart failure)
 treatment: gradual weaning from ventilator, feed and grow, avoid stress, dexamethasone may help decrease
inflammation and encourage weaning
 15% mortality in severe cases

APNEA
Definition
 absence of respiratory gas flow for 20 seconds in the preterm infant and 15 seconds in the term infant (less if
associated with bradycardia or cyanosis)
 central: no chest wall movement
 obstructive: chest wall movement continues
  mixed: combination of central and obstructive apnea
Differential Diagnosis
 apnea < 24 hrs – strongly associated with sepsis
 apnea > 24 hrs – if not pathological, apnea of prematurity
 in term infant apnea always requires full W/U CNS
 apnea of prematurity presents in the first week of life due to prematurity of CNS and resolves by 36 weeks
GA.
 seizures
 intracranial hemorrhage
 sepsis
 GI: GE reflux, esophagitis
 metabolic: low glucose, low calcium, low Na
 cardiovascular
o low and high blood pressure
o anemia, hypovolemia, PDA
 drugs: demerol, morphine
Treatment
 correct underlying cause
 tactile stimulation, reduce warming of face
 monitoring
 oxygen, CPAP, ventilation
o medications: methylxanthines (caffeine, theophylline) which stimulate CNS and diaphragm,
• S/E of caffine → tachycardia and hyponatriuemia
o doxapram (direct CNS stimulant) used in some centres

CYANOSIS OF THE NEWBORN

 central cyanosis means poor oxygenation - decreased SaO2
 decreased PaO2
 peripheral cyanosis can be normal, or it could mean sepsis,
 temperature instability, congestive heart failure, vessel
abnormalities
 Do ABGs if cyanosis seen in resting state/sleep after 30 min
of life
 SaO2 < 90% or PaO2 < 60 mmHg = emergency
 hemoglobin abnormalities cause decreased SaO2, normal
PaO2
 always check the pO2 on 100% oxygen x 10-15 min
(hyperoxic test)
o if < 100 think congenital heart disease
o if > 100 think respiratory (airway, chest, lungs),
brain or blood
Differential
 pink upper, blue lower (more common)
o PPHN
o left heart obstruction/hypoplasia
o coarctation of aorta post subclavian/interrupted aortic arch
 blue upper, pink lower
o TGA with R to L shunt across PDA

Hypoglycaemia
 True blood glucose of <2.5 mmol/L
 measured before 1 h of age for infants with the following conditions:
 o Infants of diabetic mothers, infants weighing <10th centile, prematurity, large for gestational age,
shock, seizures and infants receiving i.v. infusions.
 Risks:
o Maternal diabetes/PET o 90th percintle, <4.5kg
o Drugs o RD, Sepsis, temp
o IUGR o Symdromes
o <2.5kg, <10th percentile o Microphallus
 Causes:
o Lack substrate
o Too much insulin
o Enzyme disorders
 Clinical features
o Possibly asymptomatic
o apathy, hypotonia, poor feeding, temperature instability, apnoea, jitteriness or
convulsions
 Management
o Asymptomatic infants with a true blood glucose of 1.5–2.5 mmol/L: give early,
frequent small milk feeds at 90 mL/kg per day. If no response occurs within 2 h, give
i.v. 10% dextrose
o If the blood glucose is <1.5 mmol/L i.v. dextrose should be given. A bolus of 10%
dextrose, 2 mL/kg, should be followed by an infusion providing 5–10 mg/kg per min
of glucose. The response to therapy should be monitored by frequent blood glucose
measurements.
o Other treatments: Glucagon, diazoxide, steriods

Infection
o 0.7% newborns
o Early onset (<48hrs)
o GBS
o E.Coli
o Listeria monocytogenes
o Late onset (>48hrs)
o Coag negative Staph
o Staph A
o GBS
o Klebsiella
o Pseudomonas
o Gram negative
o Viral → HSV most serious, also RSV
o Always rule out sepsis first
o Risks
o GBS isolated
 30% carriage
 50% neonatal colonisation
o Chorioamnionitis
o Maternal fever/discagre
o Premature
o PROM
o Neurtopenia

Investigations
 Blood culture: arterial or venous, at least 2–4 mL.
 Urine: suprapubic aspiration (SPA) under ultrasound imaging if possible. If unsuccessful pass a catheter using
a 5-French gauge tube after adequate preparation of the genitalia
 o A bag collection of urine is rarely helpful
 Throat and rectal swabs and swabs of any obviously infected lesion.
 Cultures or specific PCR for viral infections.
 Lumbar puncture if meningitis is suspected. This should always be done in the presence of fever in a
newborn.
Management
 Any ill neonate should be admitted to hospital for investigation and treatment.
 Start with i.v. benzylpenicillin and gentamicin.
 Add i.v. cefotaxime if meningitis is suspected (see Antimicrobial guidelines) and i.v. metronidazole if
abdominal sepsis is suspected.
 If there is any possibility that the baby may have HSV (i.e. the mother has active genital herpes), give
aciclovir.
 Antibiotics may be subsequently tailored according to culture results
 Careful attention to temperature stability, respiratory status, fluid and electrolytes, blood pressure, blood
glucose and haematology is essential.

Neonatal rashes
 Naevus flammeus (‘stork-bite’): dilated capillaries, on the nape of the neck and on the bridge of the nose, eyelids
and adjacent forehead. They fade over 6–12 months.
 Milia: small white blocked sebaceous glands on the nose. They disappear over the first month.
 Miliaria: there are two types – ‘crystallina’ and ‘rubra’.
o Miliaria crystallina are beads of sweat trapped under the epidermis and are most prominent on the
forehead in babies who are overheated.
o Miliaria rubra, also called ‘heat rash’, usually appear after a few weeks of age, fluctuate over 2–3 weeks
and then disappear. They are related to an increasing activity of the sweat glands. They are prominent
on the face, in babies who are overheated.
 Erythema toxicum (‘toxic erythema’ or ‘urticaria of the newborn’): these are red ‘urticarial’ spots over the baby’s
trunk that peak at 2–3 days of age and generally resolve by the first week of life, but occasionally runs a
fluctuating course over a few weeks. They are harmless and of unknown cause. New lesions have a broad
erythematous base up to 2–3 cm diameter with a 1–2 mm papule or pustule.
 ‘Mongolian’ blue spot: this condition results in areas of increased melanin deposition over the lower back and
sacrum. It can be more extensive and is sometimes mistaken for bruising. It is present in most babies born to
dark skinned parents. It gradually lightens over a few years, as the rest of the skin becomes more pigmented.
 Dry skin: babies who are post-term have a thicker epidermis and hence drier-looking skin after birth. This dry
skin may occasionally crack and bleed around the hands and feet during the first few days. Emollients will help.
Otherwise the dry skin should be allowed to peel off naturally, which may take up to 1–2 months.
 Others:
o Ebsteins pearls
o Pustular melanosis
o Neonatal acne
o Sucking blister

Neonatal seizures
Clinical features
• Subtle: deviation of the eyes, staring, abnormal sucking, lip smacking or cycling movements of the limbs.
• Tonic: stiffening of limbs, frequently associated with apnoea and eye deviation.
• Clonic: movement of one or all limbs that persists despite holding the limb.
• Benign sleep myoclonus: occurs in neurologically normal infants only during sleep. The electroencephalogram
(EEG) is normal and no treatment is necessary. It can be exacerbated by benzodiazepines and usually resolves by 2
months of age.

Aetiology
Causes include:
• Hypoxic ischaemic encephalopathy: seizures occur within 24 h of the hypoxic episode, often within the fi rst 4–6 h.
• Intracranial haemorrhage (subdural, subarachnoid, intraventricular or parenchymal).
• Infection: bacterial or viral meningitis.
• Stroke.
• Metabolic:
– Hypoglycaemia.
– Hypocalcaemia/hypomagnesaemia.
– Hypo- or hypernatremia.
– Kernicterus.
– Inborn errors of metabolism (characterised by intractable seizures, with progressive loss of consciousness,
or metabolic acidosis), including:
Pyridoxine dependency.
Urea cycle disorders.
Non-ketotic hyperglycinaemia.
Fatty acid oxidation defects.
Amino acid/organic acid/hyperammonaemia.
• Drug withdrawal.
• Developmental brain abnormalities.
• Autosomal dominant neonatal seizures.
Investigations
• Bloods:
– Immediately check blood glucose.
– Electrolytes including calcium and magnesium.
– Blood gases.
– Blood cultures.
• Metabolic screen: blood ammonia, lactate, amino acids, carnitine and urinary organic acids.
• Urine analysis including ketones and reducing substances.
• Lumbar puncture and viral investigations.
• Cranial ultrasound.
• Cranial CT scan to exclude intracranial haemorrhage that may warrant neurosurgical intervention.
• MRI brain scan to determine extent of injury in hypoxic–ischaemic encephalopathy (HIE) (usually done around day
5); to exclude stroke where focal seizures are occurring, or for suspected developmental brain abnormality.
• EEG to detect seizure activity and to aid prognosis. Where conventional EEG is not immediately available, bedside
amplitude integrated EEG (aEEG) may be useful in the detection of some seizures.

Management
Admission to a NICU is mandatory for all neonates with seizures.
Attention to normoglycaemia, optimal ventilation, blood pressure control, and fl uid and electrolyte balance is
essential.
Anticonvulsants
• Phenobarbitone: 20 mg/kg i.v. over 30 min
• Phenytoin: 20 mg/kg, i.v. over 1 h.
• Clonazepam: up to 0.25 mg. This may cause apnoea. Careful monitoring and the availability of mechanical
ventilation are essential.
• Pyridoxine: 50–100 mg i.v. or p.o. should be considered for intractable seizures.
Most infants can be weaned from anticonvulsant therapy within a few days of their last seizure.

NECROTIZING ENTEROCOLITIS (NEC)

 intestinal inflammation associated with focal or diffuse ulceration and necrosis primarily affecting terminal
ileum and colon
Etiology
 multifactorial associations
 prematurity —> immature defenses
 asphyxia, acidosis and hypoxia leading to bowel ischemia
 infection: C. difficile toxin, coagulase negative staph in NICU
 hypertonic feedings / enteral alimentation
 hypovolemia, hypothermia
 milk substrate (?cow's milk protein, ?osmolality)
Clinical Features
 distended abdomen and signs of obstruction (vomiting)
  increased amount + bile stained gastric aspirate/vomitus
 frank or occult blood in stool
 feeding intolerance
 diminished bowel sounds
 signs of bowel perforation - sepsis, shock, peritonitis
Investigation
 abdomen x-ray: intramural air, perforation, fixed loops, thickened bowel wall
 high WBC, low plt, electrolyte imbalances, acidosis, hypoxia, hypercarbia
Treatment
 NPO, vigorous IV fluid resuscitation, NG decompression
 TPN
 antibiotics for infection
 serial abdominal x-rays detect early perforation
 surgery for complications (e.g. perforation)

SUDDEN INFANT DEATH SYNDROME (SIDS)

 sudden and unexpected death of an infant < 12 months of age in which the cause of death cannot be found
by history, examination and a thorough postmortem
 1-2/1,000 (leading cause of death between 1-12 months of age)
 frequency varies widely in different populations
Epidemiology
 more common in children placed in prone position (? cause vs. association)
 number of deaths peak at age 2 months
 increase in deaths during peak respiratory virus season
 most deaths occur between midnight and 8:00 am
 more common in prematurity, smoking in household, minorities, socially disadvantaged
 3:2 male predominance
 risk of SIDS is increased 3-5X in siblings of infants who have died of SIDS
Prevention
 do not place infant in prone position
 alarms/other monitors not recommended ~ increase anxiety and do not prevent life-threatening events
 avoid overheating and overdressing babies
 appropriate infant bedding

HEART MURMURS
 common in the first days of life and do not usually signify structural heart problems
 If a murmur is present at birth, it should be considered a valvular problem until proved otherwise because
the common benign transitional murmurs (eg, patent ductus arteriosus) are not audible until minutes to
hours after birth.
 If an infant is pink, well-perfused, and in no respiratory distress, with palpable and symmetrical pulses (right
brachial pulse no stronger than the femoral pulse), the murmur is most likely transitional
o . Transitional murmurs are soft (grade 1–3/6), heard at the left upper to midsternal border, and
generally loudest during the first 24 hours
 If the murmur persists beyond 24 hours of age, blood pressure in the right arm and a leg should be
determined. If there is a difference of more than 15 mm Hg (arm > leg) or if the pulses in the lower
extremities are difficult to palpate, a cardiologist should evaluate the infant for coarctation of the aorta

BIRTH TRAUMA
 Most associated with difficult delivery
o eg, large fetus, abnormal presenting
position, or fetal distress requiring
rapid extraction
  Most common injuries are soft tissue bruising, fractures (clavicle, humerus, or femur), and cervical plexus
palsies
 Skull fracture, intracranial hemorrhage (primarily subdural and subarachnoid), and cervical spinal cord injury
can also occur.
 Subgaleal hemorrhage into the large potential space under the scalp is associated with difficult vaginal
deliveries and repeated attempts at vacuum extraction
o can lead to hypovolemic shock and death from blood loss and coagulopathy triggered by
consumption of clotting factors and release of thromboplastin from the injured brain

Facial palsy
o May occur in forceps delivery
o Facial asymmetry seen when crying
o Most resolve in a few weeks-months
o If persistent after 12 months, don’t recover

Developmental dysplasia of the hips

o 1-2% some instability, 1/1000 dislocated
o 75% left hip
o Female/male
o All undergo examination birth, 6 weeks, 6months
o High risk – USS at 6 weeks
o Family history
o Breech
o Ortalani and Barlow manoeuvres to test

Neonatal abstinence syndrome

 babies born to women who are chemically dependent
 babies exhibit signs of withdrawal and may require treatment
 Do not use naloxone at delivery in these babies; it may precipitate acute withdrawal associated with
seizures.
 The onset of withdrawal is variable
o Heroin: 48–72 h.
o Methadone: may be delayed for up to 1–2 weeks.
 Withdrawal may be less evident in premature infants
 Clinical features
o Neurological signs
 Hypertonia, tremors, hyperreflexia.
 Myoclonic jerks, seizures (1–2% heroin, 7% methadone).
 Irritability, restlessness, high-pitched cry.
 Sleep disturbance.
o Autonomic system dysfunction
 Nasal stuffi ness, sneezing, yawning.
 Low grade fever, sweating.
 Skin mottling.
o Gastrointestinal abnormalities:
 Regurgitation, vomiting, diarrhoea.
o Poor feeding, dysmature swallowing.
o Failure to thrive.
o Respiratory symptoms:
 Tachypnoea.
 Increased apnoea.
o Skin excoriations (especially around the anus).
 Management of opiate withdrawal may involve the administration of morphine to assist gradual withdrawal.
List the important features in the follow-up of children born prematurely.
o Monitor growth and development
o make adjustments for the estimated due date
 correct ages for development for first 2 years
o administration of immunizations is based on the chronologic age
 Administration of hepatitis B vaccine should be delayed until the infant weighs 2,000 g
 Administration of influenza vaccine should be considered in infants with chronic medical
problems, and the pneumococcal vaccine may be beneficial at age two in children with chronic
problems, especially pulmonary disease.
o Breast-fed infants should probably receive vitamin supplements during the first year
o Screening for problems that occur more frequently in premature infants, especially vision and hearing problems.
o Screen for metabolic complications and other problems such as CP, galactosemia and PKU

What are the implications of some of these conditions for the child's future?

 Long-term neurodevelopment impairment and chronic health problems.

Neurodevelopmental outcome
 Impaired cognitive skills
 Motor deficits including mild fine or gross motor delay, and cerebral palsy
 Sensory impairment including vision and hearing losses
 Behavioral and psychological problems

Chronic health issues

o ELBW more likely to have: asthma, subnormal cognitive function and functional limitation, be enrolled in
individual education programs, and require special services.

Growth impairment
o more likely to exhibit poor growth compared to those born full-term
o lighter, shorter, and had a lower body mass index and smaller head circumference

Impairment of lung function

o reduced exercise capacity or an increase in respiratory symptoms
 o more likely to have respiratory problems as children and adults.

Insulin resistance
o More likely to have insulin resistance and higher blood pressure compared to adults born full-term

Hypertension and vascular changes

o higher blood pressure compared to those born full-term

Reproduction
o decrease reproduction in adulthood
o preterm women but not men were at increased risk of having preterm offspring.