Neonatal Flashcards
1
Q
What is Hypoxic Ischaemic Encephalopathy (HIE)?
A
Clinical manifestation of brain injury 48hrs after hypoxic event. Occurs if perinatal asphyxia has occurred (> cardiorespiratory depression)
2
Q
What is the difference between HIE and CP?
A
HIE = neonatal condition
Cerebral palsy = post-neonatal condition (i.e. severe HIE that is not tx)
3
Q
What are the causes of HIE?
A
- Placental abruption
- Shoulder dystocia > cord compression
- Maternal hypotension
- Compromised foetus (i.e. IUGR)
- Failure to breath at birth
- Hypotension > maternal haemorrhage (placental abruption, placental praevia)
- Hypertension > fulminant pregnancy-induced HTN
- Obstructed labour > malpresentation, cephalopelvic disproportion, multiple births, postmature neonates
Infants at risk > preterm, infants with CHD
4
Q
What are the S/S of HIE?
A
Response within first 48hrs grades the HIE level
Mild
- Infant irritable, responds excessively to stimulation, staring eyes, hyperventilation, hypertonia
- Complete recovery expected
Moderate
- Abnormalities of movement, hypotonic, cannot feed, has seizures
- If fully resolved by 2wks of age > good long-term prognosis
- If persistent past 2wks > bad prognosis
Severe
- No normal movements or response to pain, tone in limbs fluctuates hypo- to hypertonic, seizures prolonged and refractory to tx, multi-organ failure may be present
- 30-40% mortality
- Over 80% have neurodisability (if not cooled) > cerebral palsy
5
Q
What is the management of HIE?
A
Therapeutic hypothermia
- > 36wks, mild hypothermia can reduce morbidity from HIE
- Requires NICU
Supportive
- Resp support
- Anticonvulsants
- Fluid restriction (transient renal impairment)
- Inotropes (tx of hypotension)
- Electrolytes and glucose (tx hypoglycaemia and electrolyte imbalances)
6
Q
What is Cerebral Palsy?
A
A disorder of movement and posture due to a non-progressive lesion of the motor pathways in the developing brain.
If brain injury occurs after 2yrs > diagnosed as acquired brain injury (not CP)
7
Q
What are the causes of cerebral palsy?
A
Antenatal (80%)
- Congenital infection e.g. rubella, toxoplasmosis, CMV
- Cerebral dysgenesis/malformation
Perinatal (10%)
- HIE, birth trauma/asphyxia
Postnatal (10%)
- Intraventricular haemorrhage, meningitis, head-trauma, NAI
8
Q
What are the RFs for cerebral palsy?
A
Antenatal
- Preterm birth, chorioamnionitis, maternal infection
Perinatal
- LBW, HIE, neonatal sepsis
Postnatal
- Meningitis
9
Q
What are the S/S of cerebral palsy?
A
- Delayed milestones +/- persistent primitive reflexes
- NON-PROGRESSIVE condition so NO LOSS of previously attained milestones
- Abnormal posture / tone / gait
- Feeding difficulties > slow, gagging, vomit
- Associated non-motor problems e.g. learning difficulties, epilepsy, squints, hearing impairment
10
Q
What is the classification of cerebral palsy?
A
Spastic (70%) > MUSCLES STIFF/TIGHT
- Damage to UMNs
- Increased tone, brisk reflexes, extensor plantar/Babinski’s, ‘clasp knife’ rigidity
- Scissor gait
Subtypes:
- Hemiplegia > unilateral arm and leg, face spared
- Diplegia > legs affected to greater degree, but all 4 limbs affected
- Quadriplegia > all 4 limbs, often severe
Dyskinetic (10%) > INVOLUNTARY MOVEMENTS
- Damage to basal ganglia and substantia nigra
- Athetosis (slow, involuntary, convoluted, writhing movements of the fingers, hands, toes, and feet) / dystonia
- Random, slow, uncontrolled movements
- Repetitive and patterned movements
- Oro-motor problems e.g. drooling
- Difficulty holding onto objects due to fluctuating muscle tone
Ataxic (10%) > SHAKY / UNBALANCED
- Damage to cerebellum
- Hypotonia, ataxia of trunk and limbs, postural imbalance, intention tremor / shaky movements
- Clumsiness / instability
Mixed (10%)
11
Q
What are the investigations for cerebral palsy?
A
1st to order:
- MRI brain
Also consider:
- Assessment of hearing and vision
- EEG if seizure prone
- Bloods > FBCs, U&Es, TFTs, coagulation screen
- Metabolic screen > IEM
- XR > if severe deformity
- Gait analysis
12
Q
What is the management of cerebral palsy?
A
»MDT orientated
1st line:
- PT > encourage movement, improve strength and stop muscles from losing range of motion
- SALT > improve language abilities
- OT > identify everyday tasks that may be difficult and help make these tasks more accessible
- Consider adaptive equipment
Medical:
- Oral diazepam (good for transient use for acute spasms) / baclofen
- Botulinum toxin type A injections (for spasticity)
13
Q
What is necrotising enterocolitis (NEC)?
A
Acquired inflammatory disease affecting the gut of newborns
14
Q
What is the incidence of NEC?
A
- Most common surgical emergency in newborns
- One of the leading causes of death in prem infants
15
Q
What are the RFs for NEC?
A
- Premature
- LBW
- PDA
- Perinatal stress
16
Q
What are the S/S of NEC?
A
Onset often after initiating feeds
Early signs:
- Feeding intolerance
- Bilious vomiting
- Abdominal distension
- Bloody stools
Can quickly progress to:
- Abdominal discolouration
- Perforation
- Peritonitis
- Shock
17
Q
What are the investigations for NEC?
A
Abdominal XR:
- Pneumatosis intestinalis (intramural gas)
- Dilated bowel loops
- Portal venous gas
- Bowel wall oedema
- Pneumoperitoneum resulting from perforation
- Air both inside and outside of the bowel wall > Rigler sign
- Air outlining the falciform ligament > football sign
Bloods + culture:
- Depends on stage
- Hb low, platelets low, high WCC, metabolic acidosis, electrolyte derangement, coagulopathy
18
Q
What is the management of NEC?
A
Conservative:
- NBM + NGT decompression
- IV broad spec abx > cefotaxime/tazocin and vancomycin
- Blood and platelet transfusions
- Electrolyte imbalances correction
Surgical (if bowel perforation / necrosis):
- Resection of necrotic bowel with formation of a stoma and mucous fistula / primary anastomosis
- Extensive disease may be left as balance of resection vs short gut syndrome
- Consequences > strictures, malabsorption
19
Q
What is neonatal jaundice?
A
Excess amount of bilirubin in the neonatal circulation causing a yellow discolouration of the skin and sclera
20
Q
How common is neonatal jaundice?
A
Over 50% of newborns become visibly jaundiced
21
Q
What are the RFs for neonatal jaundice?
A
- Previous affected sibling
- Prematurity
- Breastfeeding
22
Q
What are the causes of neonatal jaundice?
A
Physiological unconjugated hyperbilirubinemia
(functional immaturity)
- Instability of foetal Hb (increased RBC volume but decreased survival)
- Defective BR metabolism / excretion in first few days of life
> > Peaks at 5d, resolves by 1-2w
Breast milk jaundice
- Only >24hrs
> > Peaks in week 2, resolves very slowly (3m)
Unconjugated haemolytic hyperbilirubinaemia
- ABO or Rh incompatibility (immune-related)
- Hereditary spherocytosis, G6PDD (defect)
- Congenital infection, bacterial sepsis (acquired)
Unconjugated non-haemolytic hyperbilirubinaemia
- Haemorrhage, polycythaemia
- Galactosaemia, hypothyroidism
Conjugated hyperbilirubinaemia
- Biliary atresia, choledochal cyst (bile duct obstruction)
- Neonatal hepatitis (congenital infection, CF, alpha-1-antitrypsin deficiency)
23
Q
What does the age of onset tell you about neonatal jaundice?
A
<24hrs = always pathological (ABO/Rh incompatibility, G6PDD, HS)
>24hrs = probably physiological (but beware sepsis and galactosaemia)
>2wks = perform prolonged jaundice screen (biliary atresia, hypothyroidism, galactosaemia, UTI, congenital infection)
24
Q
What are the S/S of neonatal jaundice?
A
- May be asx in physiological jaundice
- Visible jaundice seen at 80umol/L
- cBR > dark urine, pale stools
25
What are the S/S of kernicterus?
- Lethargy, irritability
- Poor feeding
- Increased muscle tone (arched back)
- Seizures
- Coma
> > May develop into CP (dyskinetic), learning difficulties, sensorineural deafness
26
What is kernicterus?
**uBR deposits in basal ganglia**
- Form of encephalopathy
- Causes spectrum from severe damage to death
27
What are the investigations for neonatal jaundice?
**Early jaundice (<24hrs)**
- Direct (total) serum BR
- FBC/blood film (HS), maternal and infant ABO/Rh, DAT, infection screen (blood culture, TORCH screen), G6PD levels
**Jaundice >24hrs**
- Transcutaneous BR
- If normal Hx and examination > monitor only
**Persistent jaundice (>2wks)**
- Direct and indirect serum BR
- Conjugated fraction should be obtained
- TFTs, LFTs, U&Es, DAT, G6PD levels, MC&S of blood/urine/CSF
**Conjugated hyperbilirubinaemia**
- Requires urgent investigation > USS billary tree +/- liver biopsy isotope scanning HIDA/DISIDA and referral to a paediatric liver centre
28
What is the management of neonatal jaundice?
Sometimes no treatment necessary (cBR does NOT cause harm > although cause of cBR can be bad)
**(1) Phototherapy** > *breaks down uBR so it can be excreted in urine*
- Neonate under series of lights on radiant warmer bed (eye protection, short breaks for breastfeeding, monitor temp)
- Repeat BR measurements every 4-6hrs (until drops 50 below threshold for tx)
- Use single PT unless SBR rising rapidly (>1 box per hour), SBR within 5 boxes of exchange transfusion threshold, SBR fails to respond to single PT
- +/- IVIG (used in Rh / ABO incompatibility)
**(2) Exchange transfusion + PT +/- IVIG**
- If signs of kernicterus, if intensive PT fails to lower BR, or in conjunction with PT with extremely elevated BR levels
- Give folic acid after (prevent anaemia)
29
What is transient tachypnoea of the newborn (TTN)?
**Acute, self-limiting tachypnoea in the absence of other causes**
Commonest cause of respiratory distress in term infants
30
What causes TTN?
Delayed resorption of lung fluid
31
What are the RFs for TTN?
C-section
Maternal asthma
32
What are the S/S of TTN?
*Usually occurs in first 1-3hrs following uneventful normal preterm, term vaginal or elective C-section delivery*
- Early onset tachypnoea (RR>60)
- +/- signs of respiratory distress
- Cyanosis in severe cases
- O/E = crackles, diminished breath sounds
33
What are the investigations for TTN?
**CXR:**
- Fluid in horizontal fissure
- Hyperinflation of the lungs
- Flat diaphragms
- Occasional pleural fluid
**ABG:**
- Degree of decreased pO2 > depends on amount of fluid on the lungs
**Blood cultures:**
- Exclude infectious cause
34
What is the management of TTN?
· Observation
· Supportive care
· Additional O2 if required
> > Usually settles within 1-2d
35
What is the F pneumonic for TTN?
**F**ull-term neonate
**F**irst day of life
**F**issures **F**illed with **F**luid
Di**FF**use crackles
36
What is persistent pulmonary HTN?
**The failure of the normal circulatory transition that occurs after birth**
Persistent raised pulmonary vascular resistance > R-L shunting of blood away from the lungs > life-threatening
37
What are the causes of persistent pulmonary HTN?
**Primary** = idiopathic
**Secondary** = birth asphyxia, meconium aspiration, septicaemia, RDS, diaphragmatic hernia, congenital pneumonia
38
What is persistent pulmonary HTN associated with?
· IUGR
· Foetal distress
· Down Syndrome
39
What are the S/S of persistent pulmonary HTN?
- Signs of respiratory distress
- Signs of cardiogenic shock (lethargy, hypotension, oliguria)
- Absent heart murmurs and signs of HF
> > Should be suspected in all infants when hypoxaemia is out of proportion to the severity of parenchymal lung disease on CXR
40
What are the investigations for persistent pulmonary HTN?
**CXR**
- Some pulmonary oligaemia (reduction in pulmonary volume)
**Bloods**
- Hb, WCC, glucose, clotting screen
**Echo**
- Urgent to ensure no cardiac defect
41
What is the management of persistent pulmonary HTN?
**Antenatal** *> at-risk infants should be identified*
- Correct any predisposing conditions (hypoglucaemia, polycythaemia)
**Postnatal**
- Most infants require mechanical ventilation and circulatory support
- Inhaled nitric oxide (potent vasodilator)
- Prostaglandins (keep DA patent)
- Sildenafil (viagra) has also been introduced recently
- High-frequency (oscillatory) ventilation may be useful
- For severe but reversible cases, extracorporeal membrane oxygenation (ECMO) (where the infant is placed on heart and lung bypass for several days) may be used
42
What is the prognosis of persistent pulmonary HTN?
Mortality <10%
25% likely to have some impairment such as learning difficulties / deafness
43
What is chronic lung disease of prematurity / bronchopulmonary dysplasia (BPD)?
Oxygen still required at corrected age of term with characteristic radiological changes (bronchopulmonary dysplasia)
44
What are the causes of CLD/BPD?
- Premature
- Mechanical ventilation
- Oxygen therapy - prolonged exposure to high concentrations
- Inflammation and infection - pneumonia, sepsis, RDS
- Maternal factors - smoking, infection
45
What are the RFs for CLD/BPD?
- Premature
- LBW
46
What are the S/S of CLD/BPD?
Signs of respiratory distress, poor feeding, poor weight gain
47
What are the investigations for CLD/BPD?
**CXR**
- Widespread opacification - atelectasis
- Hyperinflation and cystic changes
- Ground glass appearance
**ABG / VBG / CBG**
- Compensated respiratory acidosis reflecting chronic high pCO2
- Hypercapnia
- Hypoxia
48
What is the management of CLD/BPD?
**Respiratory support**
- Prolonged artificial ventilation
- Wean to CPAP
- Wean to additional O2
**+/- corticosteroid therapy**
- Dexamethasone for short term clinical improvement
**Prevention**
- If baby does not need intubation or ventilation, it is important to minimise ventilation-associated lung injury using strict monitoring and maintaining of tidal volume
**Long-term**
- Home O2 supported by community children’s nurses
49
What is respiratory distress syndrome (RDS)?
**Respiratory compromise in a neonate secondary to surfactant deficiency**
Surfactant deficiency > lungs more likely to collpase
50
What are the RFs for RDS?
- PREMATURITY
- Maternal diabetes
- Male
- C-section
- 2nd born premature twin
- FHx
51
How does the risk of RDS change with gestation?
**Risk decreases with gestation**
- Common if born <24w
- 50% born 26-28w
- 25% born 30-39w
52
What are the causes of RDS?
**Primary surfactant deficiency:**
- Prematurity and intrapartum hypoxia
- Acidosis
- Hypothermia
- Hypotension
**Secondary:**
- Intrapartum asphyxia
- Pulmonary infections
- Haemorrhage
- Meconium aspiration pneumonia
53
What are the S/S of RDS?
> > At delivery or within 4hrs of birth
- Early onset tachypnoea (>60)
- Respiratory distress
- Prominent grunting (baby trying to make +ve airway pressure)
- Cyanosis (if severe)
- Extremely premature infants > apnoea +/- hypothermia
- May progress rapidly to fatigue, apnoea, hypoxia
- O/E = harsh breath sounds, fine inspiratory crackles
54
What are the investigations for RDS?
**Blood gas**
- Respiratory acidosis
- Metabolic acidosis (lactic acidosis secondary to poor tissue perfusion)
- Hypoxia (right-to-left shunting)
- Hypercapnia
**CXR**
- Pneumothorax (from ventilation)
- Bilateral diffuse reticular or ‘ground-glass’ appearance
- Pulmonary oedema
- Poor lung expansion
- Indistinct heart border
**Biopsy**
- Hyaline membrane (pink) protein
**Echo**
- PDA is a complication
55
What is the management of RDS?
**Prevention / Antenatal** *> identify at-risk infants (neonatologist/NICU early involvement)*
- Amniocentesis for estimation of foetal lung maturity
- Antenatal steroids to stimulate foetal surfactant production (when preterm delivery expected <34w)
- Tocolytic therapy so steroids have at least 24hrs to work
**Postnatal**
- Surfactant replacement therapy via endotracheal tube (ETT)
- Oxygen and ventilation > CPAP, nasal cannula, conventional mechanical ventilation
- Other: High frequency oscillatory ventilation (HFOV) may be used, high flow humidified oxygen therapy may be used
**Also**
- Prophylactic abx after blood cultures
- Gentle / minimal handling
- Enteral or parenteral nutrition
56
What are the causes of pneumothorax?
- Can occur from RDS
- Can occur from the ventilation used to treat RDS
> pulmonary intestinal emphysema
57
How is pneumothorax prevented when using oxygen therapy?
Infants should be ventilated with the lowest possible pressures that provide adequate chest movement and blood gases
58
What is the management of pneumothorax?
- Immediate decompression
- O2 therapy
- Chest drain if tension pneumothorax
59
What is meconium aspiration?
Respiratory distress in the neonate secondary to the presence of meconium (foetal intestinal contents) in the trachea.
Causes mechanical obstruction and/or chemical pneumonitis > pneumonia / infection
60
When does meconium aspiration occur?
- Occurs exclusively in the immediate neonatal period
- Risk increases with increasing gestational age
61
What are the RFs for meconium aspiration?
- **GA >42wks** (44%)
- Foetal distress / hypoxia
- Maternal hx of HTN / PET / smoking / substance abuse / pre-eclampsia
- Meconium-stained amniotic fluid
- Oligohydramnios
- Chorioamnionitis
62
What are the S/S of meconium aspiration?
**Respiratory distress** > increased RR, chest retraction, recessions, grunting, overinflated chest, cyanosis (severe)
63
What are the investigations for meconium aspiration?
**CXR (diagnostic)**
- Patchy infiltrations
- Atelectasis
- Hyperinflation
- Patches of collapse and consolidation
- May show cardiomegaly, pneumothorax, pneumomediastinum
**Bloods**
- FBC, CRP, culture > rule out infection
**Blood gas**
- Reduced O2
64
What is meconium ileus?
**Bowel obstruction caused by thick, sticky meconium that has a prolonged passing time**
- Usually passes within 24hrs of delivery
- Neonate may vomit meconium instead of passing it as stool
- **Associated with CF (90%)** and biliary atresia
65
What is the management of meconium ileus?
1st line = **Gastrograffin enema**
2nd line = **Surgical decompression**
66
What is cleft lip / palate ?
Congenital malformation resulting in clefts that involve the lip, hard palate, and soft palate
67
What is the aetiology of cleft lip / palate ?
**Cleft lip** = failure of fusion of the frontonasal and maxillary processes - when the tissue that makes the lip doesn’t completely join before birth, results in an opening of the upper lip
**Cleft palate** = failure of the palatine processes and the nasal septum to fuse
68
What are the types of cleft lip / palate ?
- Combined cleft lip and palate (45%)
- Isolated cleft palate (40%)
- Isolated cleft lip (15%)
69
What are the RFs for cleft lip / palate ?
- Maternal antiepileptic and BDZ use
- Maternal smoking
- Patau's syndrome
70
What are the S/S of cleft lip / palate ?
At birth = overt clefts of the hard and soft palate
Postnatal = feeding, airway, and speech difficulties, increased risk of otitis media
71
What are the investigations for cleft lip / palate ?
- 75% detected 20w anomaly scan
- Also detected during new-born baby check
72
What is the management of cleft lip / palate ?
*MDT approach > Plastic surgeon, ENT surgeon, paediatrician, orthodontist, audiologist, SALT*
**Pre-surgical concerns:**
- Specialised feeding = slower, use of cleft palate feeders, semi-upright positioning
- Watch out for airway problems (e.g. Pierre-Robin sequence)
- Pre-surgical lip tapping, oral appliances or pre-surgical nasal alveolar moulding (PNAM) to narrow cleft
**Surgery (definitive):**
- 3m for lip
- 6-12m for palate
73
What is a diaphragmatic hernia?
Congenital defect in the formation of the diaphragm that leads to the protrusion of abdominal contents into the thoracic cavity > pulmonary hypoplasia and HTN > respiratory distress
74
When does a diaphragmatic hernia occur?
6-8wks of pregnancy (when diaphragm does not form correctly)
75
What is the most common type of diaphragmatic hernia?
**Left-sided Bochdalek hernia**
- 85%
- Part of intestine moves through left chest area > stops lungs developing properly
76
What does the prognosis of a diaphragmatic hernia depend on?
Prognosis depends on liver position and lung-to-head ratio
Only around 50% survive
77
What are the S/S of a diaphragmatic hernia?
- Concave chest at birth
- Respiratory distress in the neonate
- May be a maternal hx of polyhydramnios
Complications = intestinal obstruction, volvulus of stomach, ARDS > collapse
78
What are the investigations for a diaphragmatic hernia?
> Diagnosed on routine USS or after respiratory distress at birth
**CXR:**
- Displaced mediastinum to the L
- Collapsed L lung
- Bowel loops in thorax
**Also:**
- Blood gas
- U&Es
- Chromosomal studies
- Cardiac echo
- Renal USS
79
What is the management for a diaphragmatic hernia?
**(1)** Large NGT to decompress bowel + respiratory support
**(2)** Surgical reduction and repair > re-expansion of lung > TPN / ventilatory support during recovery
80
What is oesophageal atresia (OA)?
Malformation of the oesophagus so it does not connect to the stomach
81
What is transoesophageal fistula (TOF)?
Congenital malformation where part of the oesophagus is joined to the trachea
TOF often occurs alongside OA
82
What complications are associated with OA/TOF?
- Stomach acid can regurgitate and go into the lungs > CLD/BPD
- Polyhydramnios
- Other developmental issues
- Take longer to adjust to solids
- Tracheomalacia (collapse of the airway while breathing)
- Feeding issues > stricture formation
- GORD
83
What are the S/S of OA/TOF?
- Hx of polyhydramnios prenatally
- Excess mucus
- Coughing / choking / cyanosis with first feed
- Abdominal distension (TOF)
- Scaphoid abdomen (if OA present alone)
84
What are the investigations for TOF/OA?
*First line:*
- **Antenatal USS** > polyhydramnios (if present > MRI)
- **Antenatal MRI** > oesophageal pouch and small stomach
- **XR chest and abdomen** (obtained immediately after birth if TOF/OA suspected) > nasogastric tube coiled in upper pouch
*Consider:*
- Barium swallow
- Echo for cardiac abnormalities
85
What is the management of TOF/OA?
**Immediate = Stabilisation:**
- Infant should be placed in a slight reverse Trendelenburg position to avoid regurg / aspiration pneumonia
- NBM
- Suction catheter in upper pouch
**Surgical:**
- Surgical repair within a few days of birth
- NICU and ventilator support
86
What is biliary atresia?
Malformation of the bile ducts which causes the ducts to be abnormally narrow, blocked or absent > obstruction of bile flow > neonatal cholestasis
87
What is biliary atresia associated with?
CMV infections and aflatoxin B
88
What are the 3 types of biliary atresia?
**Type I** = common bile duct atresia
**Type II** = cystic duct atresia
**Type III** = full atresia (>90%)
89
What are the S/S of biliary atresia?
*Patients typically present in first few weeks of life with:*
- Mild jaundice (>2wks) (obstructive picture)
- Pale stools, dark urine
- NO vomiting
- Normal birth weight > faltering growth
- Appetite disturbance
- Hepatosplenomegaly
- Swollen abdomen
- Irritability
90
What are the investigations for biliary atresia?
**Raised cBR >2wks** (serum total and direct or conjugated BR)
**1st** = Abdominal USS (triangular cord sign)
TIBIDA isotope scan
**Confirmation** = ERCP +/- biopsy
**Bloods** = LFTs (raised), FBC, INR, PT, plasma or serum amino acids
**Sweat chloride test** > CF?
91
What is the management of biliary atresia?
**Surgical intervention is recommended ASAP (ideally within the first 60 days of life)**
- **1st line** = Hepatoportoenterostomy (HPE) - Kasai procedure
- **2nd line / end stage liver disease** = liver transplant
- **Ongoing** = abx prophylaxis (prevent cholangitis), nutritional support, vitamin supplementation, ursodeoxycholic acid (promotes bile flow)
92
What are the complications of biliary atresia?
- Growth failure
- Portal HTN
- Cholangitis
- Ascites
- Progressive liver disease
- Cirrhosis with eventual hepatocellular carcinoma
93
What is small bowel atresia?
Congenital malformation of the small bowel resulting in the absence or complete closure of a portion of its lumen
94
What are the 2 types of small bowel atresia?
**Duodenal atresia** = associated with polyhydramnios, Down’s, congenital cardiac abnormalities
**Duodenal stenosis** = no vomiting, potential for obstruction and ‘double bubble’ on AXR
95
What are the S/S of small bowel atresia?
- Bile-stained vomiting (jejunal or ileal atresia) > tx as intestinal obstruction until proven otherwise
- Non-bilious or bilious vomiting (duodenal atresia)
- Abdominal distension
96
What are the investigations for small bowel atresia?
**Bloods:**
- LFTs, total serum BR (uBR), INR, serum amino acids
**Urinary:**
- Organic acids, succinyl acetone, bile acids, lactate : pyruvate ratio
**AXR:**
- “Double bubble” sign
- Absent or reduced gas past the obstructions
- Dilated loops of bowel (jejunal)
97
What is the management of small bowel atresia?
**General:**
- ABCDE approach > stabilise neonate with NGT decompression + NBM
**Surgical:**
- Primary anastomosis or Ladd procedure if malrotation present
- Need to examine entire bowel to exclude other multiple atretic segments
98
What are the complications of small bowel atresia?
- Pulmonary aspiration
- Anastomotic complications (stenosis or leak)
- Proximal bowel may have abnormal peristalsis > may need prolonged post-op TPN
99
What are the S/S of urinary tract abnormalities?
**Antenatal:**
- Oligohydramnios, decreased foetal urine output
**Postnatal:**
- Irritability, infections
Also > intra-abdominal masses, haematuria, renal calculi, renal failure, HTN, hepatosplenomegaly
100
What are the investigations for urinary tract abnormalities?
- Renal USS
- DMSA scan > detect scarring and functional defects (only 2m after UTI)
- Nuclear renal imaging > assessment of kidney function and perfusion
1. MCUG > visualise anatomy, see VUR and urethral obstruction, not past infancy
2. MAG3 renogram > see MAG3 excreted from blood into urine
- IV urogram (IVG) > visualisation of majority of anomalies
- Genetic karyotyping > Potter sequence (renal problems > oligohydramnios > problems seen)
101
What is the management of urinary tract abnormalities?
**General**
- Antenatal counselling with screening for associated anomalies or kayotyping as appropriate
**Medical**
- Sx control (HTN), abx (UTI/prophylaxis), dialysis (renal failure)
**Surgical**
- Tx of cause
102
What are anorectal malformations?
Wide spectrum of congenital disorders affecting the distal anus and rectum as well as the urinary and genital tracts > all have absence of an anus in the normal position
103
What is a low anorectal anomaly?
Anus closed over, in a different position or narrower than usual, and fistula to ski
104
What is a high anorectal anomaly?
Bowel has closed end at high level, does not connect with anus, usually associated with bladder / urethral / vaginal fistula.
105
What are the S/S of an anorectal malformation?
No/delayed meconium > swollen abdomen, vomiting
If fistula > may pass stool from abnormal area
106
What are the investigations for an anorectal malformation?
Clinical, checked on neonatal check
Screen for associated abnormalities
107
What is the management of anorectal malformation?
Surgical correction by 9m (depending on specific abnormality)
108
What is cryptorchidism?
Failure of normal descent of the testicle to a scrotal position by 3m of age
109
What is a RF for cryptorchidism?
Prematurity
110
What is cryptorchidism associated with?
Patent processus vaginalis, abnormal epididymis, cerebral palsy, mental retardation, Wilms tumour, abdominal wall defects
111
What are the differential diagnoses of cryptorchidism?
Retractile testes, intersex conditions
112
What are the S/S of cryptorchidism?
- Asx
- Picked up during examination
- May have associated inguinal hernia
- Most common location of the cryptorchid testis is in the inguinal canal, may also be in the prescrotal and abdominal regions
113
What are the investigations for cryptorchidism?
**Clinical diagnosis**
- USS abdomen to aid location of the testis
- In bilateral absence, conduct hormonal tests (B-HCG) and chromosomal mapping
114
What is the management of cryptorchidism?
**Unilateral, at birth:**
- Commonly idiopathic and will resolve spontaneously
- Arrange RV for 6-8wks
- If still undescended, arrange RV at 3m
- If still undescended, refer to paediatric surgeon to be seen before 6m
**Bilateral, at birth:**
- Pituitary causes > refer to paediatricians / endocrinologists
- If concern of disorder of sexual development e.g. ambiguous genitalia or hypospadias > refer to senior paediatrician for endo and genetic ix
**Retractile** (testes have descended but come in and out of scrotal sac)
- Detect at 3m and advise annual follow-up throughout childhood
**Surgical:**
- Orchidopexy (palpable testes = inguinal approach, non-palpable = laparoscopy)
- 6-18m
**Medical:**
+/- b-hCG
**Reasons for correction:**
- Reduce risk of infertility
- Allows the testes to be examined for testicular cancer (40x more likely)
- Avoid testicular torsion
- Cosmetic appearance
115
Describe newborn resuscitation
1. Dry baby and maintain temperature
2. Assess tone, respiratory rate, heart rate
3. If gasping or not breathing give 5 inflation breaths
4. Reassess (chest movements)
5. If the heart rate is not improving and <60bpm start compressions and ventilation breaths at a rate of 3:1
116
What are the components of APGAR?
- **A** = Appearance (colour)
- **P** = Pulse (HR)
- **G** = Grimace (reflex irritability)
- **A** = Activity (tone)
- **R** = Respiratory effort
117
How is colour scored in APGAR?
2 = pink
1= body pink, extremities blue
0 = blue all over
118
How is pulse scored in AGPAR?
2 = >100
1 = <100
0 = absent
119
How is reflex irritability scored on APGAR?
2 = cries on stimulation / sneezes / coughs
1 = Grimace
0 = nil
120
How is tone scored on AGPAR?
2 = active movement
1 = limb flexion
0 = flaccid
121
How is respiratory effort scored on AGPAR?
2 = strong, crying
1 = weak, irregular
0 = nil
122
What is Caput succedaneum?
Oedema of the scalp at the presenting part of the head, typically the vertex.
123
What causes Caput succedaneum?
- Mechanical trauma of the initial portion of the scalp pushing through the cervix in a prolonged delivery
- Secondary to the use of ventouse (vacuum) delivery
124
What are the S/S of Caput succedaneum?
- Fluid collection with poorly defined margins
- Soft, puffy swelling due to localised oedema
- Crosses suture lines
125
What is the management of Caput succedaneum?
- It resolves within a few days - no intervention is necessary.
126
What is Cephalhaematoma?
A swelling on the newborns head
- Develops several hours after delivery
- Due to bleeding between periosteum and skull
- Most common site affected is parietal region
- Does not cross suture lines
127
What is the management of Cephalhaematoma?
- Managed conservatively
- May take a few months to resolve
128
What causes Cephalhaematoma?
More common following difficult / prolonged deliveries
129
What are the complications of Cephalhaematoma?
If severe - jaundice
