Neonatology (1s) Flashcards

- DDH

1
Q

What is congenital dislocation of the hip?

A
  • now known as developmental dysplasia of the hip (DDH)
  • describes spectrum of hip abnormalities
  • ranging from dysplasia (underdevelopment of joint) to subluxation through to frank dislocation
  • early recognition is vital as DDH usually responds well to conservative mx at an early stage, whereas late diagnosis is complex + often surgical mx needed
  • incidence is 1.5 per 1000 live births, girls affected 7x more commonly than boys
  • left hip is more often affected than the right + 20% of cases are bilateral
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2
Q

What is the aetiology of DDH?

A

Unclear but thought to be multifactorial:

  • genetic factors -> DDH tends to run in families, inheritable features contributing to hip instability are generalised joint laxity + a shallow acetabulum
  • hormonal changes -> changes occurring in late pregnancy may aggravate ligamentous laxity in infants
  • post-natal factors -> neonatal positioning of child may contribute to DDH
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3
Q

What are risk factors for DDH?

A
  • female
  • first born
  • positive family history
  • breech presentation at delivery
  • maternal oligohydramnios (due to restriction of intrauterine foetal movement)
  • associated neuromuscular disorders eg. cerebral palsy
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4
Q

What is the clinical presentation of DDH?

A
  • asymmetry of skin folds around hip (unless bilateral DDH)
  • painless limp (unless in adolescence or adulthood)
  • limited abduction of the hip
  • swayback walk
  • leg length discrepancy
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5
Q

It is ideal to diagnose every case of DDH at birth. Screening forms part of the examination of the newborn ad 6-8 wk old babies. Screening takes the form of Barlow and Ortolani tests.

What is the Barlow test?

A
  • stabilise the non-test hip
  • for the side being tested -> adduct hip + push femur into acetabulum
  • this will dislocate a dislocatable hip, giving a “click” + moving the hip posteriorly
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6
Q

What is the Ortolani test?

A
  • starts in the position where a hip has been dislocated
  • stabilise pelvis by firmly holding symphysis pubis + coccyx between thumb + mid finger
  • then, w/ baby’s hips + knees flexed, and the index finger of examiner’s other hand on the greater trochanter -> abduct baby’s hip
  • a palpable ‘clunk’ signifies relocation of a posteriorly dislocated hip
  • infants w/ positive test should have hip USS + urgent review in a neonatal or specialist hip clinic for follow-up
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7
Q

Treatment for DDH depends upon the age at which the diagnosis is made.

What is the treatment for a newborn?

A
  • can be corrected conservatively by putting baby in double nappies or by using a harness that holds hips abducted + flexed
  • a padded Von Rosen splint can be used for babies up to 3 months old
  • but Pavlik Harness should be used for older infants who would otherwise be able to crawl out of a Von Rosen splint
    • if worn correctly for roughly 12 wks, a stable hip will be achieved
    • following removal of splint, hip is examined radiologically or with USS to ensure that hip is reduced
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8
Q

DDH: What is the treatment for age group of 6 months old to 6 years old?

A
  • the hip must be reduced + held in this position until acetabular development is adequate
  • closed reduction is performed gradually over 3 wks as manipulation under anaesthesia carries a high risk of avascular necrosis
  • traction applied to both legs + abduction increased until legs widely separated
  • if reduction achieved -> legs are splinted in plaster cast in the flexed, abducted + slightly internally rotated position
  • if closed reduction unsuccessful -> formal open reduction in theatre may be required
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9
Q

DDH: What is the treatment for older than 6 years old?

A
  • unilateral DDH is usually managed w/ open reduction, sometimes combined w/ a corrective osteotomy of femur
  • bilateral disease results in symmetrical deformity + therefore less noticeable
  • surgical management carries greater risk as failure on one side results in asymmetrical deformity
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10
Q

What is the prognosis like for DDH?

A
  • without treatment, DDH results in progressive deformity + disability
  • increased risk of secondary osteoarthritis
  • earlier the treatment, more likely the child will develop a normal (or near normal) hip
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11
Q

Jaundice is the most common condition in newborns that requires medical attention. How common is neonatal jaundice?

A
  • about 50-70% of term babies + 80% of preterm babies develop jaundice in the first week of life
  • jaundice usually appears 2-4 days after birth + resolves 1-2 wks later without need for treatment
  • most cases physiological
  • jaundice in the first 24 hrs of life is considered pathological
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12
Q

Describe bilirubin metabolism

A

Newborns have higher bilirubin level than expected in adult population, due to foetal Hb gives RBCs shorter lifespan of 90 days vs 120 days for adults. They have immature livers + reduced elimination of bilirubin due to low volume of colostrum babies receive in first 24-48hrs of life.

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13
Q

Physiological jaundice is usually noted at postnatal day 2, peaks on days 3 to 5, and then decreases. Serum bilirubin levels up to 205.2 micromol/L (12 mg/dL) are considered physiological in term neonates.

What are the causes of physiological jaundice?

A
  • increased bilirubin load secondary to increased RBC volume, decreased RBC lifespan or increased enterohepatic circulation
  • decreased uptake by liver bc of decreased ligandins or binding of ligandins to other anions
  • reduced conjugation in liver bc of reduced UDPGT activity
  • reduced excretion into bile
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14
Q

Jaundice can be categorised in terms of time of presentation.

What are the causes if jaundice is presenting within 24 hours of birth?

A
  • Haemolytic disease (Unconjugated)
    • rhesus incompatibility
    • ABO incompatibility
    • G6PD deficiency
    • hereditary spherocytosis
  • Conjugated
    • ​congenital infection -> sepsis
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15
Q

What are the causes of jaundice presenting between 24hrs and 2 weeks?

A
  • breastfeeding jaundice -> unclear why, resolves without mx or complication, mothers should be encouraged to continue to breastfeed
  • physiological jaundice
  • polycythaemia
  • infection eg UTI
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16
Q

What are the causes of prolonged jaundice (not fading after 14 days in term babies, or 21 days in prems)?

A
  • Unconjugated:
    • physiological or breast feeding
    • UTI
    • haemolytic disease
    • congenital hypothyroidism
    • cystic fibrosis
  • Conjugated:
    • bile duct obstruction (eg. biliary atresia)
      • most important condition to exclude + requires surg mx
      • presence of pale stools + dark urine implies limited passage of bile into gut
    • neonatal hepatitis
17
Q

If there are still signs of jaundice after 14 days, a prolonged jaundice screen is performed, including what?

A
  • total serum bilirubin levels -> conjugated or unconjugated
  • coombs test -> ABO or Rh
  • TFTs
  • FBC, haematocrit, reticulocytes, blood groups
  • urine MC+S + reducing sugars
  • U+Es + LFTs
18
Q

What is the management for neonatal jaundice?

A
  • plot bilirubin measurements on graph of bilirubin against age in hours to determine when + whether phototherapy or exchange transfusion is required
  • the treatment threshold is lower for preterms
  • phototherapy most common treatment
  • can use exchange transfusion
  • supportive: hydration, albumin tranfusion, IV IG
19
Q

What is phototherapy?

A
  • uses light energy to convert bilirubin to soluble products (lumirubin + other isomers)
  • can be excreted without conjugation
  • efficacy depends on irradiance - so exposing baby will lead to more rapid reduction in serum bilirubin, as will using light from above and below
  • breast feeds should be brief to maximise time under lights
  • SE: eye damage, diarrhoea, separation from mother, fluid loss
  • intense phototherapy is an adjunct to exchange transfusion
  • refer to NICE / local trust guidelines for thresholds
20
Q

What is exchange transfusion?

A
  • uses warmed blood (37oC)
  • 160 mL/kg (double volume)
  • given ideally via umbilical vein IVI with removal via umbilical artery
  • the aim is to remove bilirubin in those with severe or rapidly rising hyperbilirubinaemia
21
Q

What is kernicterus?

A
  • encephalopathy caused by deposition of unconjugated bilirubin
  • neurotoxic effects of bilirubin may be transient or permanent
  • clinical features:
    • lethargy, poor feeding, hypertonicity, opisthotonus (back arching due to muscle spasm), shrill cry, seizures
  • risk increased w/ extremely high bilirubin levels (360umol/L; lower in prems)
  • long-term sequelae incl athetoid movements, deafness, cerebral palsy + reduced IQ
  • prevented by photoherapy +/- exchange transfusion
22
Q

Respiratory distress syndrome refers to lung disease caused by surfactant deficiency. What is the pathophysiology of this?

A
  • surfactant is a mixture of proteins (10%) + phospholipids (90%) excreted by type II pneumocytes in the alveolar epithelium
  • it keeps the alveoli open by increasing alveolar surface tension
  • it is first produced by the foetus at 34wks
  • in RDS, there is deficiency of surfactfant, hence -> alveolar collapse + inadequate gas exchange
23
Q

What are the risk factors for respiratory distress syndrome?

A
  • born before 34wks
    • 91% if born at 23-25wks
    • 52% if 30-31 wks
  • caeserian delivery
  • hypothermia
  • perinatal hypoxia
  • meconium aspiration
  • maternal diabetes
  • past family history
  • males
  • 2nd twin
24
Q

What is the clinical presentation of respiratory distress syndrome?

A

Signs of respiratory distress develop within 4 hours after delivery:

  • tachypnoea (>60/min)
  • grunting
  • nasal flaring
  • intercostal recession
  • cyanosis
  • CXR -> diffuse granular patterns (ground glass appearance) +/- air bronchograms
25
Q

How is respiratory distress syndrome prevented?

A
  • mothers expecting to deliver prematurely given corticosteroids antenatally
  • help promote maturity of type 2 alveolar cells
  • betamethasone/dexamethasone, 2 doses, 12hrly given to mother 1-7 days before birth
  • last dose given at least 24hrs before birth
26
Q

What is the management for RDS?

A
  • delay clamping of cord to promote placento-fetal transfusion
  • give oxygen via an oxygen-air blender, start w/ 21% + increase if no improvement in HR, despite good chest movement
  • attach oxymeter + follow local guidelines for sats
  • sats of 85% are normal in first 5-10mins of life if baby active
  • if not, inc O2 by 10% every minute until improving
  • if spontaneously breathing, stabilise w/ CPAP
  • if gestation <27wks -> intubate + give prophylactic surfactant via ET tube +/- 2 further doses if ongoing O2 demand/ventilation requirement
  • aim for sats 85-93%
  • wrap up warmly + take to NICU/SCBU incubator
  • if any deterioration check DOPE: displaced ET tube, Obstructed (secretions, blood), Pneumothorax, Equipment failure (ventilator, tubing)
  • Abx -> give penicillin + gentamicin until congenital pneumonia excluded
  • fluids + nutrition -> 10% glucose IVI, inositol essential nutrient promoting surfactant maturation + helps reduce complications
    • full parenteral nutrition can be started day 1
    • minimal enteral feeding w/ expressed breast milk can also be started on day 1
27
Q

What are the complications of respiratory distress syndrome?

A
  • pulmonary haemorrhage or infection
  • persistent pulm hypertension of newborn
  • pneumothorax caused by artifical ventilation
  • bronchopulmonary dysplasia caused by pressure + volume trauma caused by artifical ventilation, oxygen toxicity + infection
  • death
  • intraventricular haemorrhage + patent ductus arteriosus
28
Q

What is prematurity and how common is it?

A
  • infant born before 37 weeks gestation
  • 7% of births
29
Q

What are the risk factors for prematurity?

A
  • placental - placenta praevia, abruption, insufficiency
  • uterine - malformation, cervical incompetence
  • maternal - pre-eclampsia, chronic illness, infection, smoking, drug abuse, BV, GD, underweight or obese
  • foetal - distress, multiple preg, chromosomal abnormality, infection
  • other - premature rupture of membranes, trauma, iatrogenic, prev pre-term birth
30
Q

Outline problems associated in various systems in the very premature infant

A
  • Resp - RDS, bronchopulmonary dysplasia, pneumothorax, apnoea
  • CVS - patent ductus arteriosus, hypotension
  • GI - jaundice, necrotising enterocolitis, inguinal hernia, feed intolerance
  • Neuro - intraventricular haemorrhage, ischaemic brain injury, hydrocephalus, retinopathy, sensori-neural deafness
  • Haem - anaemia of prematurity, imapired leucocyte function
  • Metabolic/Endo - hypothermia, hypocalcaemia, hyponatraemia, hypoglycaemia, osteopenia
  • Infection - septicaemia, meningitis, UTI, fungal/viral infections
  • Social - parental anxiety + distress, family relationship disruption
31
Q

What is the long-term outlook for very premature babies?

A
  • survival under 23 wks gestation is v rare
  • neurodevelopment problems include cognitive delay, seizures, educational difficulties, behavioural problems, cerebral palsy
  • those requiring artifical ventilation @ 36wks -> bronchopulm dysplasia (chronic lung disease of prematurity) -> at risk of developing chest infections, given monoclonal antibody to resp syncitial virus
  • retinopathy + blindness occurs in 20% of v low weight preterms
  • sensorineural hearing loss common
  • iron supplementation required up to 6 months corrected age to prevent anaemia
  • those born after 32wks -> excellent prognosis
32
Q

Discuss the ethics of interventions in the extremely premature

A
  • ​neonate born <24wks or less than 500g has a negligible chance of surivival
  • parents do not always receive sufficient counselling during an emergency admission
  • so aren’t well-informed to accept withdrawal of treatment or quality of life decisions
  • prospective parents not educated earlier in pregnancy about extreme prem delivery
  • crucial info explaining neonatal issues only offered to labouring women during emergency admission
  • most have difficulty understanding risks and benefits of baby’s treatment
  • parents confronted by ethical decision on whether to continue treatment or not
33
Q

What is meant by ‘small for gestational age’?

A
  • babies below the 10th centile on the growth chart for their gestational age
  • they may be genetically small or have experienced IUGR
34
Q

What is meant by intrauterine growth restriction (IUGR)?

A
  • the baby is small for their gestational age AND appears thin + malnourished
  • something has prevented the foetus from reaching its genetic growth potential
35
Q

What are the causes of IUGR?

A
  • Maternal:
    • undernutrition
    • maternal hypoxia: cyanotic heart disease; chronic resp disease
    • drugs: alcohol, cigarettes, illicit drugs
  • Placental:
    • reduced vascular supply: pre-eclampsia, HT, diabtes, renal disease
    • thrombosis or infarction: sickle cell, anti-phospholipid syndrome
    • sharing: multiple foetuses
  • Foetal:
    • chromosomal disorders or syndromes
    • structural
    • congenital infection
36
Q

What is asymmetrical IUGR?

A
  • commonest form of IUGR
  • caused by uteroplacental insufficiency late in pregnancy
  • baby’s weight or height is in a lower centile than the head circumference bc the vital organ growth eg. the brain, is spared at the expense of the liver glycogen stores + subcutaneous fat
    • example is maternal pre-eclampsia
  • infants rapidly put on weight after birth
37
Q

What is symmetrical IUGR?

A
  • caused by prolonged period of poor intrauterine growth
  • normally due to foetal factors
  • head circumference + body weight lie in similar centiles
  • these infants are likely to remain small
38
Q

What are complications of IUGR?

A
  • intrauterine hypoxia + death + perinatal asphyxia
  • monitoring of foetus is required to assess if + when an elective caeserean needs to be done
  • neonatal problems:
    • hypothermia due to relatively large SA compared to mass
    • hypoglycaemia due to lack of fat + glycogen stores
    • hypocalcaemia
    • polycythaemia
  • later life: evidence IUGR associated w/ HT, DMT2, CHD + stroke