Paeds - neonatology (0-finals)

Question 1

Why is compliance necessary? Explain the 2 ways surfactant helps.

Answer 1

Surfactant is produced by Type 2 alveolar cells between week 24-34.

It stops the alveoli from collapsing in on themselves by reducing the surface tension of the fluid of the lungs (stops the water molecules from pulling towards each other).

Therefore the surfactant keeps the alveoli inflated, maximising their surface area.

Increased compliance is when less force is required to expand the alveoli/ lungs during inspiration.

Surfactant also ensures the equal expansion of alveoli during inspiration.

When an alveoli expands, the surfactant molecules become more thinly spread which allows surface tension to increase again.
Therefore already expanded alveoli dont really expand more, allowing other alveoli to expand more.

Question 2

Describe the cardio/ resp changes that happen during birth.

Answer 2

• thorax is squeezed as body passes through vagina, clearing fluid from lungs
• first breath is stimulated by birth/temp change/sound/physical touch
  
  • adrenaline and cortisol are also released in response to labour stress, stimulating resp effort
  • function of first breath is to expand collapsed alveoli for the first time
  • This causes a drop in pulmonary vascular resistance
• Decrease in pulmonary vascular resistance causes a drop in RA pressure
  
  • the LA pressure is greater than the RA pressure, causing a functional closure of the foramen ovale
  • the foramen ovale then structurally closes = fossa ovalis
• Increased blood oxygenation causes a drop in circulating prostaglandins
  
  • this causes closure of the ductus arteriosis = ligamentum arteriosus
• When umbilical cord is clamped and blood stops flowing through umbilical veins, ductus venosus stops functioning
  
  • ductus venosus structurally closes a few days later = ligamentum venosum
    *

Question 3

What are the risks and benefits of delayed umbilical cord clamping?

Answer 3

Benefits = delay clamping for at least 1 min in uncompromised babies

• placental transfusion! allows more foetal blood from placenta to enter the babys circulation
• improves Haemoglobin, iron stores, BP
• reduces risk of intraventricular haemorrhage and necrotising enterocolitis

Risk = babies that require neonatal resus should be clamped asap so resus team can work

• increase in neonatal jaundice (needs phototherapy)

Question 4

What score would you use in neonatal resuscitation?

Answer 4

APGAR score

Question 5

What are the 7 things you need to do for the baby immediately after birth?

Answer 5

• clamp the umbilical cord
• dry the baby
• keep the baby warm with a hat and blankets
• skin to skin
• vitamin K
• label the baby
• measure the weight and length

Question 6

Why and how would you give vitamin K?

Answer 6

• babies are born with a vit K deficiency
• it will help prevent bleeding, particularly intracranial/ umbilical stump/ GI bleeding
• you generally give it as an IM injection in the thigh shortly after birth (helpful as also stimulates the baby to cry and expand its lungs)
• can also give it orally however takes longer to work (give at birth, 7 days, 6 weeks)

Question 7

When would you initiate breast/ bottle feeding?

When would you have the first bath?

Answer 7

Feeding = as soon as baby is alert enough!

Bath = waituntil the baby is warm and stable. no issue waiting a few days!

Question 8

What 3 examinations would you have to think about after leaving the delivery room?

Answer 8

• Newborn examination within 72 hours
• Blood spot test
• Newborn hearing test

Question 9

What does the blood spot screening test for?

Answer 9

It is taken on day 5 with parents consent. (Day 8 latest)

• heel prick for 4 separate drops of blood
• takes 6-8 weeks to see results

Screens for 9 congenital conditions:

• Sickle cell disease
• Cystic Fibrosis
• Congenital hypothyroidism
• Phenylketonuria
• Medium-chain acyl-CoA dehydrogenase deficiency (MCADD)
• Maple syrup urine disease (MSUD)
• Isovaleric acidaemia (IVA)
• Glutaric aciduria type 1 (GA1)
• Homocystinuria (HCU)

Question 10

You do the newborn examination within 72 hours of birth. When would you repeat it?

Answer 10

6-8 weeks by the GP

Question 11

Newborn Examination - How would you measure the O2 sats?

Answer 11

• Measure pre-ductal saturations in the baby’s right hand
  
  • RH gets blood from R Subclavian Artery, a branch of the brachiocephalic artery (which branches from aorta before ductus arteriosus)
• Measure post-ductal saturations from either foot
  
  • feet receive blood from descending aorta (occurs after the ductus arteriosus)
• Normal saturations are 96% or above
• Ductus arteriosus stops functioning 1-3 days after birth
• Until then, it allows blood from deoxygenated R sided circulation to mix with oygenated L sided circulation after the lungs
• Certain congenital heart conditions are duct dependent (rely on the mixing of blood across the ductus arteriosus), and when the DA closes there can be a rapid deterioration
  
  • therefore you can pick these up by measuring the difference in pre-ductal and post-ductal saturations

Question 12

Newborn Examination - What would you look out for on examination of the head?

Answer 12

• size, shape, dysmorphology, caput succedaneum (swelling or oedema on scalp after delivery), cephalohaematoma (monitor for jaundice and anaemia), any facial injuries, etc
• occipital frontal circumference of head
• anterior and posterior fontanelles
• Sutures eg. overlapping sutures (typically resolve as baby grows)
• Ears = skin tags, low set, asymmetry
• Eyes = squints can be normal, epicanthic folds (Down’s), purulent discharge (infection)
• Red reflex using ophtalmoscope (check for symmetry) = absent with congenital cataracts and retinoblastoma
• Mouth = cleft lip or tongue tie
• little finger in mouth for suckling reflex, palpate all the way back to check for cleft palate

Question 13

Newborn examination - What would you look for on the shoulders and arms?

Answer 13

• shoulder symmetry = check for clavicle fracture
• arm movements = check for erbs palsy
• brachial and radial pulses
• palmar creases = single palmar crease can be associated with Down’s
• Digits = number, straight, curved (clinodactyly)
• sats probe on right wrist for pre-ductal reading

Question 14

Newborn examination - what would you look for in the abdomen?

Answer 14

• shape of abdomen = concave abdo can indicate diaphragmatic hernia with abdo contents in the chest
• umbilical stump = look for discharge, infection, periumbilical hernia
• Palpate for organomegaly, hernias, masses

Question 15

Newborn Examination - what would you look for in the genitals on a newborn exam?

Answer 15

• observe for sex, ambiguity, abnormalities
• palpate testes and scrotum = check both are present and descended, check for hernias and hydroceles
• inspect penis = hypospadias, epispadias, urination
• inspect anus = patent?
• ask about meconium = has the baby opened their bowels?

Question 16

Newborn Examination - What would you look for in a babys legs and back on the newborn examination?

Answer 16

Back

• inspect and palpate spine = curvature, spina bifida, pilonidal sinus

Legs

• observe legs and hips = equal movement, skin creases, tone and talipes
• Barlows and Ortolani manoeuvres = checks for clunking, clicking, dislocation of hips
  
  • if clunking/clicking of hips = refer for hip US to rule out developmental dysplasia of hips
• Count toes

Question 17

Newborn Examination - what are some reflexes that you would try to elicit in a newborn examination? (5)

Answer 17

• Moro reflex = arms and legs extend when baby is rapidly tipped backwards
• Suckling reflex = finger in the mouth will prompt them to suck
• Rooting reflex = tickling cheek will cause them to turn towards stimulus
• Grasp reflex = finger in their palm will cause them to grasp
• Stepping reflex = when held upright with their fee touching a surface, they will make a stepping motion

Question 18

Newborn Examination - What are these skin findings that may be seen on a newborn?

Answer 18

haemangiomas

Question 19

Newborn Examination - What are these skin findings that may be seen on a newborn?

Answer 19

erythema toxicum

Question 20

What is talipes?

Answer 20

• clubfoot
• where the ankles are in supinated position (rolled inwards)
• positional talipes = muscles are tight around the ankle
  
  • bones unaffected so foot can still be moved into normal position via physiotherapist and simple exercises
• structural taplies = involves bones of foot and ankle
  
  • requires referral to orthopaedic surgeon

Question 21

What condition are port wine stains associated with?

Answer 21

Sturge-Weber syndrome

can have visual impairment, learning difficulties, headaches, epilepsy, glaucoma

Question 22

Where would you document the newborn examination?

Answer 22

• on the Newborn and Infant Physical Examination (NIPE) computer system
• baby’s Red Book

Question 23

Birth Injuries - What is caput succedaneum?

Answer 23

• fluid (oedema) collecting on the scalp outside the periosteum
• due to pressure to a specific area of the scalp during traumatic/ prolonged/ instrumental delivery
• fluid is outside of periosteum so can cross suture lines
• usually resolves within a few days

Question 24

Birth injuries - What is cephalohaematoma?

Answer 24

• Also described as “Traumatic Subperiosteal Haematoma”
• due to damage to the blood vessels during a traumatic/ prolonged/ instrumental delivery
• collection of blood between the skull and the periosteum
• As it is below the periosteum, the lump does not cross the suture lines of the skull (unlike caput succedaneum)
• the blood also can cause discolouration of the skin in the affected area (unlike caput succedaneum which will have no or only mild discolouration)
• Monitor as there is a risk of jaundice or anaemia (blood that builds up in the haematoma can break down and release bilirubin)

Question 25

Birth Injuries - what method of delivery would increase the risk of facial paralysis?

Answer 25

Forceps delivery is associated with facial nerve injury.

Function normally returns spontaneously within a few months, otherwise requires neurosurgical input

Question 26

What is Erbs palsy and how would it happen in birth?

Answer 26

• injury of C5/C6 nerves in the brachial plexus!
• results in weakness of shoulder abduction and external rotation, arm flexion and finger extension = “Waiter’s Tip”
  
  • internally rotated shoulder
  • extended elbow
  • flexed wrist facing backwards (pronated)
  • lack of movement in affected arm
• Associated with shoulder dystocia, traumatic/ instrumental delivery, large birth weight
  *

Question 27

Birth Injuries - How would you diagnose a clavicle fracture and how would it happen in birth?

Answer 27

• confirmed with US or Xray
• presents on newborn exam with:
  
  • noticeable lack of movement in affected arm
  • asymmetry of shoulders (affected shoulder lower than normal shoulder)
  • pain and distress on movement of the arm
• associated with shoulder dystocia, traumatic/instrumental delivery, large birth weight
• Management = immobilisation of arm (conservative)
• Risk = injury to brachial plexus and nerve palsy

Question 28

What are some common organisms to think about when thinking about neonatal sepsis?

Answer 28

• Group B Streptococcus (GBS) = especially if there is maternal vaginal GBS colonisation during pregnancy
• Escherichia coli
• Listeria
• Klebsiella
• Staphylococcus aureus

Question 29

What are some risk factors for neonatal sepsis?

Answer 29

• vaginal GBS colonisation
• GBS sepsis in previous baby
• maternal sepsis, chorioamnionitis or fever >38C
• Prematurity <37 weeks
• early (premature) rupture of membranes
• prolonged rupture of membranes (PROM)

Question 30

What are some features and red flags of neonatal sepsis?

Answer 30

Clinical features:

• fever
• redued tone and activity
• poor feeding
• resp distress or apnoea
• vomiting
• tachycardia or bradycardia
• hypoxia
• jaundice within 24 hours
• seizures
• hypoglycaemia

Red flags

• confirmed or suspected sepsis in mother
• signs of shock
• seizures
• term baby needing mechanical ventilation
• resp distress starting more than 4 hours after birth
• presumed sepsis in another baby in a multiple pregnancy

Question 31

How would you manage presumed sepsis?

Answer 31

Initial…

• If there is one risk factor OR clinical feature, monitor obs and clinical condition for at least 12 hours
• Blood cultures before antibiotics
• Check FBC and CRP
• Lumbar Puncture if infection is strongly suspected or there are features of meningitis eg. seizures
• If there are 2 or more risk factors OR clinical features, start antibiotics
  
  • also start antibiotics if there is 1 red flag
  • give antibiotics within 1 hour of deciding to start them
  • 1st line = benzylpenicillin and gentamycin
    
    • (cefotaxime or a 3rd gen cephalosporin is an alternative in lower risk babies)

Later…​

• Blood cultures should be back in 36 hours
• Check CRP again in 24 hours
• Stop antibiotics if baby is clinical well, blood cultures are -ve 36 hours later & both CRP results are <10
• Consider an LP if any CRP is >10
• Check CRP again in 5 days if still on treatment
• Stop antibiotics if CRP is normal in 5 days, baby is clinically well, and LP and blood cultures are -ve

Question 32

What is Hypoxic-Ischaemic encephalopathy (HIE) and when would you suspect it?

Answer 32

When there is prolonged or severe hypoxia during birth which leads to ischaemic brain damage.

Permanent damage can result in cerebral palsy, severe cases can result in death.

You would suspect it in any neonates who have experienced…

• events that could lead to hypoxia in the perinatal or intrapartum period
• acidosis on the umbilical abg
• poor Apgar scores
• features of HIE
• evidence of multi organ failure

Question 33

What are some causes of HIE?

Answer 33

Anything that can lead to asphyxia to the brain can cause HIE:

• maternal shock
• intrapartum haemorrhage
• prolapsed cord which can cause compression of the cord during birth
• nuchal cord (cord wrapped around babys neck)

Question 34

What is the staging for HIE and name some mild features

Answer 34

Sarnat Staging

Mild features of HIE:

• poor feeding
• generally irritable and hyper-alert
• resolves within 24 hours
• normal prognosis

Question 35

Name some moderate features of HIE

Answer 35

• poor feeding, lethargic, hypotonic, seizures
• Can take weeks to resolve
• up to 40% develop cerebral palsy

Question 36

Name some features of severe HIE

Answer 36

• reduced consciousness, apnoeas, flaccid and reduced or absent reflexes
• up to 50% mortality
• up to 90% develop cerebral palsy

Question 37

How would you manage hypoxic-ischaemic encephalopathy?

Answer 37

• supportive care eg. neonatal resus, ventilation, nutrition, acid base balance, treatment of seizures, circulatory support
• Therapeutic Hypothermia
  • baby is transferred to neonatal ICU and cooled using cooling blankets and a cooling hat
  • temp targeted between 33-34C measured using a rectal probe
  • Continue cooling for 72 hours, then gradually warm baby back up to normal temp over 6 hours

Goal of therapeutic hypothermia is to reduce inflammation and neurone loss after acute hypoxic injury.

Reduces risk of cerebral palsy, developmental delay, LD, blindness and death.

Question 38

What is meconium aspiration syndrome?

Answer 38

It is a spectrum of disorders marked by various degrees of resp distress in the new born infant following aspiration of Meconium Stained Amniotic Fluid, which can happen either antenatally or during birth.

This happens when the meconium is released in utero, instead of after birth.

MSAF happens as a result of in-utero peristalsis in term or post term infants, resulting in meconium passage.

This peristalsis can be the result of foetal hypoxic stress or vagal stimulation due to cord compression.

Some evidence of chronic hypoxia causing peristalsis too, which also leads to foetal gasping which results in Meconium Aspiration Syndrome.

Once Meconium Stained Amniotic Fluid is aspirated, it causes vasoactive and cytokine substances to be released in the baby that activate inflammatory pathways and also inhibit the effect of surfactant in the lungs.

Question 39

Describe the pathophysiology of partial or total airway obstruction once Meconium Stained Amniotic Fluid is aspirated by the foetus

Answer 39

• Meconium is thick and sticky so aspiration can result in total or partial mechanical airway obstruction.
• This can result in atelectasis and a “ball-valve” effect with air trapping
• once airways are obstructed, pulmonary pressure increases
  
  • this leads to Right to Left shunt (through DA or FO) resulting in V:Q mismatch, worsening foetal hypoxia

Question 40

Describe the pathophysiology of foetal hypoxia once Meconium Stained Amniotic Fluid is aspirated by the foetus

Answer 40

• previously mentioned airway obstruction resulting in increase of pulmonary vascular pressures resulting in R to L shunt, causing V:Q mismatch
• meconium contains pro-inflammatory cytokines eg. TNF and interleukins that can cause airway oedema and surfactant inactivation
  
  • surfactant inactivation results in increased surface tension of alveoli which reduces efficiency of gas exchange

Question 41

What is persistent pulmonary hypertension and why does it happen in meconium aspiration syndrome?

Answer 41

Results from remodelling of the pulmonary vascular bed in response to hypoxia, vasoactive mediators in the meconium and ventilation/perfusion mis-match.

Question 42

What are some of the risk factors for Meconium Aspiration Syndrome?

Answer 42

Conditions that cause foetal hypoxia and foetal gasping will increase the risk of aspirating meconium

• gestational age >42 weeks
• foetal distress (tachycardia/ bradycardia)
• Intrapartum hypoxia secondary to placental insufficiency
• thick meconium particles
• Apgar score <7
• Chorioamnionitis +/- prolonged pre-rupture
• Oligohydramnios
• In utero growth restriction
• maternal htn, dm, pre-eclampsia, eclampsia, smoking, drug abuse

Question 43

What are some clinical features of Meconium Aspiration Syndrome?

Answer 43

Non specific presentation of resp distress

• tachypnoea (RR>60 bpm)
• Tachycardia (>160 bpm)
• Cyanosis
• grunting
• nasal flaring
• recessions (intercostal, supraclavicular, tracheal tug)
• hypotension (systolic bp <70mmHg)

+ hx of risk factors + confirmed presence of meconium in the amniotic fluid and aspirated meconium (clinical signs of post-maturity and MSAF staining)

Question 44

How would you investigate meconium aspiration syndrome?

Answer 44

• chest xray will show:
  
  • increased lung volumes
  • asymmetrical patchy pulmonary opacities
  • pleural effusions
  • pneumothorax or pneumomediastinum
  • multifocal consolidation due to chemical pneumonitis
• FBC, CRP, Blood cultures
• ABG
• Dual pulse oximetry (right upper limb and either lower limb for pre and post ductal)
• Echocardiography to exclude congenital heart abnormalities causing the pulmonary htn
• cranial US to assess any hypoxic damage to the brain

Question 45

How would you manage Meconium Aspiration Syndrome?

Answer 45

• observation, especially o2 sats
• place newborns under an infant warmer as hypothermia inhibits surfactant production
• blood glucose, U&Es, fbc, crp, ca2+ should be assessed and corrected if necessary
• IV fluids, then NG or oral feeds if permitting
• nasal cannula to achieve o2 sats of 92-97%
  
  • CPAP can be started with nasal prongs in newborn with spontaneous breathing and good resp effort
  • If mechanical ventilation is required, refractory pulmonary hypertension can occur which should be treated with inhaled Nitric Oxide
• Antibiotics if clinical suspicion of infection
• bolus of surfactant in newborns with moderate MAS or pneumothorax
  *

Question 46

What are some complications of Meconium Aspiration Syndrome?

Answer 46

• Inflammation process predisposes foetal lung to increased risk of infection and can casue a chemical pneumonitis
• thick meconium can cause a “ball-valve” effect. air leaks can happen due to alveolar hyperdistension resulting in pneumothorax or pneumomediastinum
  
  • air leaks are at a higher risk when mechanical ventilation is used
• PPHN
• MAS can lead to cerebral hypoxia resulting in cerebral palsy
• Chronic lung disease can develop from barotrauma and oxygen toxicity

Question 47

What is physiological jaundice in a newborn?

Answer 47

• there is a high conc of RBC in the foetus and neonate and they are fragile. Their liver function is also less developed (unconjugated bilirubin is conjugated in the liver)
• Normally the bilirubin is excreted via the placenta, but at birth the placenta is removed.
• So there is a normal rise in bilirubin shortly after birth = mild yellowing of skin/sclera from 2-7 days of age.
• Usually resolves by 10 days
• Jaundice in the first 24 hours of life is pathological!!!

Question 48

What are some causes of neonatal jaundice (increased production of bilirubin)?

Answer 48

• haemolytic disease of the newborn
• ABO incompatibility
• Haemorrhage
• Intraventricular haemorrhage
• cephalo-haematoma
• polycythaemia
• sepsis and disseminated intravascular coagulation
• G6PD deficiency

Question 49

What are some causes of neonatal jaundice (decreased clearance of bilirubin)?

Answer 49

• Prematurity
• breast milk jaundice
• neonatal cholestasis
• extrahepatic biliary atresia
• endocrine disorders (hypothyroid and hypopituitary)
• Gilbert syndrome

Question 50

What is a complication that is at higher risk with jaundice in premature neonates?

Answer 50

• physiological jaundice is more exagerated in premature babies as their livers are less mature
• increased risk of complications like kernicterus
• kernicterus = brain damage due to high bilirubin levels
  
  • bilirubin crosses the blood-brain barrier and can cause direct damage to the CNS
  • CNS damage is permanent = cerebral palsy, LD, deafness
  • baby presents as less responsive, floppy, drowsy baby with poor feeding

Question 51

How does haemolytic disease of the newborn cause jaundice in the neonate?

Answer 51

• incompatibility between rhesus antigens on the surface of the RBC of the mother and foetus
• When a woman is rhesus D negative and her baby is rhesus D positive, the mother’s immune system will produce antibodies to the rhesus D antigen (stimulated by her babys blood mixing into her bloodstream)
• The mother has become sensitised to Rhesus D antigens
• When she has her next pregnancy, her anti-D antibodies can cross the placenta into the foetus.
• If the baby is rhesus Positive, these antibodies can cause haemolysis of their RBCs = anaemia + high bilirubin levels

Question 52

When is jaundice “prolonged” and what does this mean?

Answer 52

Jaundice is prolonged when it lasts longer than would be expected in physiological jaundice.

• >14 days in full term babies
• >21 days in premature babies

Past these times, you would look for conditions that would cause jaundice to persist after the initial neonatal period

Eg. biliary atresia, hypothyroidism, G6PD deficiency

Question 53

How would you investigate neonatal jaundice?

Answer 53

• FBC and blood film for polycythaemia or anaemia
• conjugated bilirubin (high = hepatobiliary cause)
• blood type testing of mother and baby for ABO or rhesus incompatibility
• Direct Coombs Test for haemolysis
• Thyroid Function (for hypothyroid)
• Blood and urine cultures (infection)
• Glucose-6-phosphate-dehyrogenase levels for G6PD deficiency

Question 54

How would you manage neonatal jaundice?

Answer 54

• monitor bilirubin levels on treatment threshold charts (age of the baby on the X axis, total bilirubin level on the y axis) to know when to commence treatment
• Phototherapy is usually adequate to correct neonatal jaundice
  
  • converts unconjugated bilirubin into isomers that can be excreted in the bile and urine (does not require conjugation in the liver)
  • use a light-box to shine blue light, make sure to put eye patches on to protect babys eyes
  • monitor bilirubin throughout treatment and rebound bilirubin should be measured 12-18 hours after stopping to make sure they do not rise
• If extremely high levels, you can use exchange transfusion

Question 55

What is considered an apnoea?

Answer 55

When breathing stops spontaneously for more than 20 seconds,

or shorter periods with o2 desturation or bradycardia

Question 56

What are some causes of apnoea in neonates?

Answer 56

immaturity of the autonomic nervous system (controls RR and HR)

Apnoeas can also indicate developing illness eg.

• infection
• anaemia
• airway obstruction (can be positional)
• CNS pathology eg. seizures, haemorrhage
• gastro-oesophageal reflux
• neonatal abstinence syndrome

Question 57

How would you manage apnoea in neonates?

Answer 57

• premature babies are attached to apnoea monitors in neonatal units that make a noise when apnoea is occuring
• tactile stimulation can be used to prompt baby to restart breathing
• IV caffeine can be used to prevent apnoea and bradycadria in babies with recurrent epidodes

Question 58

What is the pathophysiology of retinopathy of prematurity?

Answer 58

• typically affects babies born before 32 weeks gestation
• Retinal blood vessel development starts at around 16 weeks and is complete by 37-40 weeks
• blood vessel growth is stimulated by hypoxia (normal condition in the retina during pregnancy)
  
  • when the retina is exposed to higher o2 conc in a preterm baby, esp when supplementary o2 is given during medical care, the stimulant for normal blood vessel development is removed
• When hypoxic environment recurs, retina responds by producing excessive blood vessels (neurovascularisation) + scar tissue
  
  • the abnormal blood vessels may regress and leave the retina without blood supply
  • the scar tissue can cause retinal detachment

Question 59

When would you screen for retinopathy of prematurity

Answer 59

Screening

• babies born before 32 weeks or under 15 kg are screened for ROP by an opthalmologist
  
  • starts at 30-31 weeks gestational age in babies born before 27 weeks
  • starts at 4-5 weeks of age in babies born after 27 weeks
  • screening should happen every 2 weeks until the retinal vessels enter zone 3 (usually at around 36 weeks gestation)

Question 60

How would you describe and assess the disease in ROP?

Answer 60

Retina is divided into 3 zones

• zone 1 = optic nerve and macula
• zone 2 = from the edge of zone 1 to ora serrata (the pigmented border between the retina and ciliary body)
• zone 3 = outside the ora serrata

When describing the diseased areas, describe it in stages on a clock face:

• stage 1 (slightly abnormal vessel growth) - stage 5 (complete retinal detachment)
• eg. disease is from 3 to 5 oclock

Use the term “plus disease” to describe additional findings

eg. tortuous vessels, hazy vitreous humour

Question 61

How would you manage retinopathy of prematurity?

Answer 61

You systematically target areas of the retina to stop new blood vessels developing

• 1st line = transpupillary laser photocoagulation (to halt and reverse neovascularisation)
• Others =
  • cryotherapy
  • injections of intravitreal VEGF inhibitors
  • Surgery if retinal detachment occurs

Question 62

What is Respiratory Distress Syndrome and why does it happen?

Answer 62

• affects premature neonates (born before lungs produce adequate surfactant)
• inadequate surfactant leads to high surface tension in alveoli
• results in atelectasis (lung collapse) as it is more difficult for the lungs to expand
• leads to inadequate gaseous exchange = hypoxia, hypercapnia, resp distress
• CXR shows “ground glass” appearance

Question 63

How would you manage resp distress of the newborn?

Answer 63

• antenatal steroids eg. dexamethasone is given to mothers with suspected or confirmed pretern labour
  
  • increases production of surfactant

Premature neonates may need…

• intubation and ventilation to assist breathing if resp distress is severe
• endotracheal surfactant (artificial surfactant delivered to lungs via endotracheal tube)
• continuous positive airway pressure via nasal mask to help keep lungs inflated whilst breathing
• supplementary o2 to maintain o2 sats between 91-95% in preterm neonates

Question 64

What is necrotising enterocolitis?

Answer 64

a disorder affecting premature neonates, where a part of the bowel becomes necrotic.

Necrotic bowel can lead to bowel perforation, which can result in peritonitis and shock.

Question 65

What are some risk factors for developing necrotising enterocolitis?

Answer 65

• low birth weight or very premature
• formula feeds
• resp distress and assisted ventilation
• sepsis
• patient ductus arteriosus and other congenital heart disease

Question 66

How would necrotising enterocolitis present?

Answer 66

• intolerance to feeds
• vomiting, particularly with green bile
• generally unwell
• distended, tender abdo
• absent bowel sounds
• blood in stools
• in perforation = peritonitis, shock, severely unwell

Question 67

How would you investigate for necrotising enterocolitis?

Answer 67

• FBC for thrombocytopaenia and neutropenia
• CRP for inflammation
• capillary blood gas to show a metabolic acidosis
• blood culture for sepsis
• abdo xray in supine position (lateral and lateral decubitus views are also useful)
  
  • dilated loops of bowel
  • bowel wall oedema (thickened bowel walls)
  • pneumatosis intestinalis (gas in the bowel wall)
  • pneumoperitoneum (free gas in the peritoneal cavity indicates perforation = Riglers sign)
  • gas in the portal veins
  • air outlining falciform ligament = football sign

Question 68

How would you manage necrotising enterocolitis?

Answer 68

• nil by mouth with IV fluids, total parenteral nutrition, antibiotics
• NG tube can be used to drain fluid and gas from stomach and bowels
• Surgical emergency!!
  
  • Risks = short bowel syndrome after surgery, long term stoma, strictures, abscess formation, etc.

Question 69

What kind of substances would cause neonatal abstinence syndrome?

Answer 69

• opiates
• methadone
• benzodiazepines
• cocaine
• amphetamines
• nicotine
• cannabis
• alcohol
• SSRI antidepressants

Question 70

When would you see withdrawal symptoms from

a) opiates, diazepam, SRI, alcohol

and

b) methadone, benzodiazepines

Answer 70

a) 3-72 hours after birth
b) between 24 hours and 21 days after birth

Question 71

Name some CNS, vasomotor/ resp, metabolic and GI withdrawal symptoms you would see?

Answer 71

CNS = irritability, increased tone, high pitched cry, not settling, tremors, seizures

Vasomotor = yawning, sweating, unstable temp, pyrexia

Resp = tachypnoea

Metabolic/ GI = poor feeding, regurg or vomiting, hypoglycaemia, loose stools, sore nappy area

Question 72

How would you manage Neonatal Abstinence Syndrome?

Answer 72

• put an alert in mothers notes who are known to use substances, so after birth the neonate can be monitored more for NAS and managed
• Babies are kept in hospitals with monitoring on a NAS chart for atlesat 3 days (48 hours for SSRI) to monitor for withdrawal symptoms
• A Urine sample is taken from neonates to test for substances
• Support neonates in a quiet, dim environment with gentle handling and comforting

Medicine for severe symptoms (gradually wean them off)

• opiate withdrawal = oral morphine sulphate
• non-opiate withdrawal = oral phenobarbitone

Question 73

What additional things would you consider in NAS?

Answer 73

• test for Hep B, C, and HIV
• safeguarding and social service involvement
• safety net advice for readmission if withdrawal signs and symptoms occur
• folow up from paeds, social services, health visitors and GP
• support for mother to stop using substances
• check suitability for breastfeeding in mothers with substance use

Question 74

What is sudden infant death and when can it happen?

Answer 74

Sudden unexplained death in an infant, usually within the first 6 months of life.

Risk factors:

• prematurity
• low birth weight
• smoking during pregnancy
• male

Question 75

How would you minimise the risk of Sudden Infant Death Syndrome?

Answer 75

• put baby on their back when not directly supervised
• keep head uncovered
• place feet at foot of the bed to stop them sliding down and under the blanket
• keep cot clear of lots of toys/ blankets
• comfy room temp of 16-20C
• avoid smoking or handling baby after smoking (smoke stays on clothes)
• avoid co-sleeping
• if co-sleeping, avoid alcohol, drugs, smoking, sleeping tablets, deep sleepers

Question 76

How would you support families suffering from SIDS?

Answer 76

• the lullaby trust is a charity that supports families affected
• bereavement services and counselling
• Care of Next Infant (CONI) Team supports parents with their next infant after SID.
  
  • extra support and home visits, resus training, access to equipment like movement monitors that alarm if baby stops breathing for a prolonged period

Question 77

What is foetal alcohol syndrome? + clinical features

Answer 77

When mum drinks alcohol in pregnancy, alcohol can cross the placenta and enter the foetus and affect foetal development.

Effects are greatest in the first 3 months of pregnancy.

Presents as:

• Microcephaly
• Thin upper lip
• Smooth flat philtrum (groove between nose and upper lip)
• Short palpebral fissure (short horizontal distance from one side of the eye and the other)
• LD
• behavioural difficulties
• hearing/vision problems
• cerebral palsy

Question 78

What is congenital rubella syndrome and how does it present in a baby?

Answer 78

Caused by maternal infection with Rubella Virus during pregnancy.

Risk is highest during first 3 months of pregnancy.

Presents with:

• congenital cataracts
• congenital heart disease (PDA, Pulmonary Stenosis)
• Learning Disability
• Hearing Loss

Question 79

How would you try and prevent Congenital Rubella Syndrome?

Answer 79

• women planning to become pregnant should ensure they have had the MMR vaccine
  
  • if in doubt, test for Rubella Immunity
  • If they do not have rubella antibodies, they can be vaccinated with 2 doses of the MMR 3 months apart
• Pregnant women should not receive the MMR vaccine as it is a live vaccine!
  
  • non-immune women should be offered the vaccine after birth

Question 80

Why is Varicella Zoster Virus exposure dangerous in pregnancy?

Answer 80

• causes more severe cases in the mother eg. Varicella pneumonitis, hepatitis, encephalitis
• Can cause Foetal Varicella Syndrome
• Can cause severe neonatal varicella infection if mum is infected around delivery

Question 81

How would you manage and avoid varicella zoster exposure in pregnancy?

Answer 81

• mums who have previously had chickenpox are immune and safe
  
  • if in doubt test IgG levels for VZV
  • If mum is not immune, offer varicella vaccine before or after pregnancy
• If mum is exposed to chickenpox during pregnancy, check immunity
  
  • if she is not immune, treat with IV varicella immunoglobulins as prophylaxis against developing chickenpox
    
    • Give within 10 days of exposure
• If the chickenpox rash starts in pregnancy, treat with oral aciclovir if they present within 24 hours and are more than 20 weeks gestation

Question 82

When would congenital varicella syndrome occur and how would it present?

Answer 82

Occurs in around 1% of chickenpox cases in pregnancy.

Typically occurs when there is infection in first 28 weeks of gestation.

Clinical features:

• foetal growth restriction
• microcephaly, hydrocephalus, learning disability
• scars and significant skin changes following Dermatomes
• Limb hypoplasia (underdevelopment)
• Cataracts and inflammation in the eye (chorioretinitis)

Question 83

How would congenital cytomegalovirus present?

Answer 83

• foetal growth restriction
• microcephaly
• hearing loss
• vision loss
• learning disability
• seizures

Most cases of CMV in pregnancy dont cause congenital cmv

Question 84

What is Congenital Toxoplasmosis and how does it present?

Answer 84

• infection with the Toxoplasma gondii parasite (usually asymptomatic) during pregnancy
• Risk is higher later in the pregnancy
• spread by contamination with faeces from a cat that is a host of the parasite

Congenital Toxoplasmosis presents with a classic triad:

• Intracranial calcification
• Hydrocephalus
• Chorioretinitis

Question 85

How would congenital Zika syndrome present?

Answer 85

zika virus is spread by host Aedes mosquitos

congenital zika syndrome presents with:

• microcephaly
• foetal growth restriction
• intracranial abnormalities like ventriculomegaly and cerebellar atrophy

Question 86

Newborn Examination - What are these skin findings that may be seen on a newborn?

Answer 86

Port wine stain

Question 87

Newborn Examination - What are these skin findings that may be seen on a newborn?

Answer 87

Mongolian Blue Spot

Question 88

Newborn Examination - What are these skin findings that may be seen on a newborn?

Answer 88

Cradle Cap

Question 89

Newborn Examination - What are these skin findings that may be seen on a newborn?

Answer 89

Desquamation

Question 90

Newborn Examination - What are these skin findings that may be seen on a newborn?

Answer 90

Milia

Question 91

Newborn Examination - What are these skin findings that may be seen on a newborn?

Answer 91

Naevus simplex (stork bite)

Question 92

Newborn Examination - What are these skin findings that may be seen on a newborn?

Answer 92

Transient Pustular Melanosis

Question 93

What are 3 neural tube defects?

Answer 93

• Spina bifida occulta
  
  • absent posterior vertebral arch hasnt fused
  • no neuro deficit
  • presents with hairy patch on skin, growth and cord tethering
• Meningocoele
  
  • herniation of pia and arachnoid filled with CSF
  • no neuro deficit
• Myelomeningocoele
  
  • usually open with exposed meninges and leaking CSF
  • neuro deficits eg. motor/sensory loss in LL, neuropathic bladder and bowel
  • typically gets scoliosis and hydrocephalus due to Arnold-Chiari malformation (herniation of cerebellar tonsils thorugh foramen magnum)
  • surgery prevents infection but cannot restore function

Question 94

What is exomphalos (ompalocoele)

Answer 94

When abdo viscera (covered in peritoneum) are herniated out of the umbilical ring.

Mostly identified by routine foetal anomaly scans and raised afp (also high in duodenal atresia and congenital nephrosis)

Associated with Beckwith-Wiedemann syndrome, Downs syndrome, cardiac and kidney malformations

Management:

• caesarian section to reduce risk of sac rupture
• staged repair!!
  
  • primary closure may be difficult due to lack of space/ high intra-abdo pressure