Neonatology Flashcards
1
Q
What is the perinatal mortality
A
Stillbirths + deaths within 7 days of birth per 1000 deliveries
2
Q
In toxoplasmosis what is the relationship between infection, severity and gestation
A
Lower gestation- reduced risk infection, increased severity
Older gestation- 60% risk of transmission, reduced severity
3
Q
What are the signs of congenital toxoplasmosis
What is the treatment
A
Chorioretinitis
Hydrocephalus, microcephaly
Diffuse cerebral calcifications
Hepatospenomegaly
Jaundice
Thrombocytopenia
Pyrimethamine and Sulfadiazine
Leucovorin- helps prevent bone marrow suppression
If during pregnancy in first trimester, Sulfadiazine only
2nd trimester Sulfadiazine and Pyrimethamine
4
Q
What are the signs of congenital syphilus
A
“SYPHILIS”
-Sniffles
-Yucky skin
-Periosteal reaction
-Hepatosplenomegaly
-iyes (“eyes”: chorioretinitis, glaucoma
-Lymphadenopathy
-mIscarriage
-Saddle shaped nose
4
Q
What are the signs of congenital syphilus
A
“SYPHILIS”
-Sniffles
-Yucky skin
-Periosteal reaction
-Hepatosplenomegaly
-iyes (“eyes”: chorioretinitis, glaucoma
-Lymphadenopathy
-mIscarriage
-Saddle shaped nose
5
Q
What does VDRL stand for and what does it test
A
Veneral disease related lab test - checks for syphilis antibodies
6
Q
What are the symptoms of congenital Rubella
A
RUBELLA
-Retina- cataracts, retinopathy
-U= heart= PDA, PA stenosis, PV stenosis
-Blueberry rash
-Ears- SNHL
-Little- SGA
-Lagging- neurodevelopment delay
-A bit liver and spleen - hepatosplenomegaly
7
Q
What are the symptoms of congenital CMV
What is the treatment
A
CCMMVV
-Calcifications- periventricular
-Chorioretinitis
-Milestone delay- CP and reduced IQ
-Microcephaly
-Very poor hearing- SNHL
-Very big liver - Hepatosplenomegaly
-Blueberry rash
6 weeks oral Valganciclovir
8
Q
What are the signs of congenital HSV infection
What is the treatment in
-primary infection
-secondary infection
-pregnancy
A
Scarring
microcephaly
Choriretinitis
Primary infection- IV Acyclovir
2nd infection- swab at 24-48 hours- if positive commence IV Acyclovir
Pregnancy: 4 weeks acyclovir before delivery and C-section
9
Q
What are the signs of congenital varicella syndrome
When is the baby most at risk
When is neonatal varicella contracted
What is the treatment
What is the prevention
A
Skin: Scarring
Limbs: hypoplasia, parasthesia
CNS: microcephaly, brain aplasia, hydrocephalus
Eyes: chorioretinitis, cataracts
5 days before to 2 days post birth
Treatment: IV Acyclovir
Prevention: VIG 5 days before - 2 days post delivery
10
Q
What are the symptoms of withdrawal
What is the most common sx
What percentage of babies exposed to heroin experience withdrawal vs how many require treatment
A
Wakefullness
Irritability
Tremors
Hypertonia, high pitched cry
Diarrhoea
Rhinnorhea
Autonomic instability- fevers, tachycardia
Weight loss and poor feeding
Apnoea and respiratory distress
Lacrimation
Common - tremors
Least common- seizures - 2%
70% experience withdrawal
only 1/2 of those need treatment
11
Q
What is the normal range of amniotic fluid
What aneuploidy causes oligohydramnios
What aneuploidies cause polyhydramnios
A
5-25cm
T13
T18, T21
12
Q
What is the criteria for fetal hydros
What is an immune causes
What are some non-immune causes
A
Subcutaneous oedema AND 2 of
-pericardial effusion
-pleural effusion
-ascites
Immune- Alloimmune haemolytic disease of the newborn
Non-immune- high cardiac output States
-anaemia e.g. Twin-twin-transfusion syndrome
-Cardiac: SVT, cardiomyopathy
-GI: diaphragmatic hernia
-Chromosomal- T21, Turners
-Infection: parvovirus
-pulmonary lymphangiectasis –> chylothorax
13
Q
What is the difference between IUGR and SGA
A
IUGR= deviation from expected growth pattern - due to unfavourable uterine conditions that cause change in fetal growth pattern
SGA= birth weight <10th centile- can be normal or pathological
14
Q
What is the most accurate way of measure fetal age in first and second trimester
A
Crown-rump length before 12 weeks-most accurate
Biparietal diameter 2nd trimester = after 30 weeks accuracy falls to +/-3 weeks
15
Q
What are the 8 cardinal movements of the foetus during delivery
A
1- Head floating not engaged
2- Engagement and flexion
3- Further descent and internal rotation
4- Complete rotation so posterior of head is aligned along the pubic symphisus
5- Complete extension
6- Restitution- external rotation
7- Delivery anterior shoulder
8- Delivery posterior shoulder
15
Q
What are the 8 cardinal movements of the foetus during delivery
A
1- Head floating not engaged
2- Engagement and flexion
3- Further descent and internal rotation
4- Complete rotation so posterior of head is aligned along the pubic symphisus
5- Complete extension
6- Restitution- external rotation
7- Delivery anterior shoulder
8- Delivery posterior shoulder
16
Q
What makes up fetal Hb
What direction is the fetal Hb oxygen saturation curve shifted
What does this mean
At what age do you start making adult Hb
What age is most fetal Hb removed
A
Fetal Hb- 2-alpha and 2-Gamma Hb molecules
Fetal Hb has higher affinity for oxygen, shifting the curve to the left and having a steeper curve
This means that despite reduced overall oxygen content, the Hb is as saturated as adult Hb
Third trimester
Most removed by 3-6 months; All removed by 1 year
17
Q
What percentage of fetal circulation is delivered to the lungs
What cases pulmonary vasoconstriction
A
10%
Hypoxia, Hypercapnia, Acidosis
18
Q
What are a premature infants insensible losses
What are their Na, K and calcium requirements/ day
A
2-3ml/kg/hr
3mmol/kg/day
2mmol/kg/day
1mmol/kg/day
19
Q
What does SMOF stand for
A
Soy bean
MCFA
Olive oil
Fish oil
20
Q
What is the most common cause of hyperkalaemia in a premature infant
A
Non-oliguria hyperkalaemia due to immature Na/K ATPase
21
Q
What causes anaemia in a premature infant
A
reduced iron stores
prematurity suppresses erythropoiesis
rapid expansion of blood volume with growth
frequent sampling
22
Q
What babies receive supplemental iron
A
<37 weeks
<2500g
23
What babies receive probiotics
<32 weeks and/or <1500g until 36 weeks gestation
Must be mixed with feeds
24
what is the earliest marker of metabolic bone disease
when do you start measuring
Serum phosphate (+ ALP)
4-6 weeks
25
What is the NICU survival rate for the following ages
-24
-25
-26-27
70%
80%
85%
26
What are the 3 biggest causes of morbidity in NICU
Respiratory distress
Sepsis
IVH
27
Name bacteria that are considered coagulase negative staph
How does it normally present in the neonate
S. epidermis
S. haemolyticus
S. saprophyticus
Present: sepsis/bacteraemia
TX: IV Vancomycin
28
Where is listeria acquired from
What are maternal sx
What is seen in neonatal infection
What is the treatment
Soft cheese, pate, uncooked meats, coleslaw
Maternal flu like illness in pregnancy
Baby
-maculopapular rash
-transplacental transmission --> disseminated abscess --> multiorgan dysfunction
-important cause of purulent meningitis
TX: IV Amoxicillin and Gent
NOT cephalosporins
29
What are the layers of the scalp
Subcutaneous tissue
Connective tissue
Aponeurosis
Loose connective tissue
Periosteum
30
Where is the default/ blood loss in
-cephalohaematoma
-subgaleal
-caput seccundum
-subperiosteal
-sub-galeal-aponeurosis - between the periosteum and overlying galeal aponeurosis
-tissue swelling in the connective tissue layer
31
What amount of blood loss is required to cause an increase in head circumference by 1cm
What is the transfusion order in a smbgaleal haemorrhage
35mls of blood is needed to increased HC by 1cm
1) RBC - FFP - RBC - Cyroprecipitate
2) calcium/glucose infusion
3) RBC- FFP- RBC-PLT-Calcium
32
In a subgaleal what do you give if the pH is <7.3
Sodium bicarbonate
33
What is used in the treatment of clavicle fractures
Supportive cares only
-PRN paracetamol
-Pin sleeve to clothes to immobilise the arm
34
What position is the arm held in during and what nerve roots are affected in
-Erbs palsy
-Klumpe palsy
-Facial nerve palsy
Erbs = waiters tip position- C5/C6 affected = adducted arm, extended at elbow, pronated, internally rotated
Klumpke palsy = C7=C8=T1 affected = wrist drop, claw hand, Horners syndrome
Facial nerve palsy = compression of lower motor neurons of facial nerve during delivery or with forceps. Ipsilateral facial paralysis including the forehead
35
What are the embryology stages of lung development
Embryonic stage- 4-7 weeks - lung bud formation from endoderm
Pseudoglanular stage 7-17 weeks- development of airways from bronchi --> terminal bronchioles plus main pulmonary artery
Canullicular phase 17-24 weeks- lengthening of the airways, development of type 1 and type 2 pneumocystis, surfactant production at 24 weeks
Saccular phase- 24-36 weeksdifferentiation of type 1 and type 2 pneumocytes, progressive surfactant production, proliferation of alveolar ducts
Alveolar phase- 36 weeks =40 weeks - expansion of gas exchange area, maturation of nerves and capillaries
36
What type of alveolar cells produce surfactant
Type II pneumocystes
37
What is the main composition of surfactant
Phospholipids and lipids
38
What is the main constituent of exogenous surfactant preparations
Dipalmitoyl-phosphatidylcholine (DPPC)
39
What lipids make up surfactant and what is their role
Lipids facilitate in the spread, recycling and adsorption of surfactant. They also have immune function
Surfactant protein A- immune
Surfactant protein B- adsorption and recycling - deficiency is fatal
Surfactant protein C- adsorption and recycling - deficiency results in progressive lung fibrosis
Surfactant protein D- immune
40
What is the cause of neonatal
hypoxia
hypercapnia
V/Q mismatch as alveolar collapse due to reduced compliance and increased chest elastic recoil
Hypercapnia - small tidal volume, reduced minute volume
41
What age bracket should be provided antenatal steroids
Gestation 23-34+6
42
How do antenatal steroids work
Increase the differentiation between epithelial cells into type 1 and 2 pneumocysts
43
what factors do antenatal steroids reduce
RDS
NEC
IVH
Mortality
44
what makes up exogenous surfactant
DPPC
Surfactant proteins B + C
45
Who should receive surfactant
FiO2 >30% and clinical diagnosis of RDS i.e. those premature infants with CPAP failure - <32 weeks, FiO2 >30%, on CPAP since birth
OR
Clinical RDS and deteriorating in respiratory status
46
LISA is associated with what 3 better outcomes than INSURE surfactant delivery and in WHAT GESTATION
BPD, Pneumothorax, IVH
29-32 weeks
47
What has LISA been associated increased risk of in extremely preterm infants
Spontaneous intestinal perforation
48
What is the definition of chronic lung disease
Ongoing oxygen requirement at 36 weeks (if born <32 weeks) with persistent oxygen for >28 days
OR
If born >32 weeks, persistent oxygen requirement after 28 days
49
What is the new vs old causes of CLD
OLD: overly aggressive mechanical ventilation and oxygen toxicity --> airway inflammation, injury and parenchymal fibrosis
NEW: Infants born <1500g or <28 weeks have significant disruption in lung development resulting in:
= large abnormal alveoli --> less surface area for gas exchange
= abnormal vasculature with thickening of muscular layer --> pulmonary HTN
50
97% of cases of CLD occur below what age
28 weeks
51
BPD in relation to mechanical ventilation is primarily due to what type of trauma
Volutrauma
52
What are 5 risk factors for developing CLD
-Prematurity (especially <28 weeks) - lungs in the saccular phase of development with few alveoli and poorly organised support structures
-Mechanical ventilation - volutrauma
-Oxygen toxicity
-Infection e.g. chorioretinitis
-PDA
52
What are 5 risk factors for developing CLD
-Prematurity (especially <28 weeks) - lungs in the saccular phase of development with few alveoli and poorly organised support structures
-Mechanical ventilation - volutrauma
-Oxygen toxicity
-Infection e.g. chorioretinitis
-PDA
53
In chronic lung disease why are steroids not used as treatment
When are they considered
They do provide benefit of weaning off ventilation
BUT
They increase the risk of neurodevelopment outcome adversity e.g. CP and having increased risk of GI bleed/perforation, hyperglycaemia, poor growth
Considered
-7-21 days requiring mechanical ventilation
-In supplemental oxygen
-High risk of CLD
54
What is the difference in length between a low dose course, high dose 'Cummings course" and tailored course of steroids
Low dose course- 10 days
High dose course-= 43 days
Tailored coures = 18 days
55
What are the treatment of CLD
Supportive
-Optimise ventilation to reduce volutrauma and oxygen toxicity
-Fluid restrict to 150ml/kg/day
-Fortify to ensure adequate nutrition and growth
Consider diuretics if concerns regarding pulmonary oedema (PDA, excessive weight gain) OR if fluid restriction has failed to wean off the ventilator
Routine steroids NOT recommended as worse neurodevelopment outcome AND GI perforation
RSV prophylaxis
56
What are babies with CLD at increased risk of
-death in first year of life
-reactive airway disease
-pulmonary HTN
-neurodevelopmental impairment
-steroid use --> osteopenia
57
Define an apnoea of prematurity
Cessation of breathing for >20 seconds plus associated bradycardia (<2/3 baseline) OR desaturation <80% AND infant <37 weeks gestation
58
What is the most common type of apnoea in a premature infant
Mixed central and obstructive- starts obstructive then becomes central
59
Why do premature infants get Apnoea of prematurity
Immaturity of the central respiratory drive
-Blunted response to hypercapnia or central and peripheral chemoreceptors
-Biphasic response to hypoxia - initial hyperopnoea then hypopnea and apnoea
PLUS
Immature and floppy airway with impaired protective reflexes
60
What is an example of a Methyxanthines and when are they used
Caffeine
Apnoea of prematurity
61
How does caffeine work
It reduces the respiratory drive threshold to hypercapnia
62
What has caffeine been shown to reduce the incidence of
BPD
ROP
Cerebral palsy
Post natal steroid use
Cognitive delay at 18-21 months
63
what is the MoA of Doxapram
Stimulates peripheral chemoreceptors in the carotid bodies of the carotid arteries
63
what is the MoA of Doxapram
Stimulates peripheral chemoreceptors in the carotid bodies of the carotid arteries
64
Why does meconium aspiration cause respiratory distress
Causes physical obstruction- ball valve mechanism with hyperinflation
Chemical pneumonitis
Displaces surfactant
65
What do you see on oximetry in a baby with PPHN
>10mmHg difference in saturation between pre and post ductal saturations
Represents ongoing persistence of fetal circulation with R --> L shunting across the PDA due to ongoing high respiratory vascular resistance
66
What are the 3 pathophysiological causes of NEONATAL PPHN
-Vasoconstriction- failure to normally respond to reduced hypoxia and improved ventilation
-Obstruction causes increased pulmonary venous pressures
-Abnormal pulmonary vasculature with increased medial muscle thickness- due to fetal hyoxia OR due to pulmonary hypoplasia
67
How is pulmonary vascular resistance calculated
PVR = (Pressure Pulmonary Artery - Pressure Pulmonary vein) / Pulmonary blood flow
68
What grades of mean pulmonary arterial pressure are norma, mild, moderate and severe PPHN
normal <25
mild 25-40
moderate 40-55
severe >55
69
What pulmonary pressures compared to systemic pressures correlate to normal, mild pphn, moderate pphn and severe pphn
o Normal: PA pressure <1/3 systemic pressure
o Mild: PA pressure 1/3-2/3 systemic pressure
o Moderate: PA pressure 2/3 – 1 systemic pressure
o Severe: PA pressure >1 systemic pressure
70
what is the MoA of inhaled NO
Diffuses into vascular endothelial cells exclusively and activated cGMP-dependent protein kinases. These protein kinases then phosphorylate various ionic channels in the Endoplasmic reticulum causing calcium sequestration. This prevents calcium mobilization in the cell resulting in relaxation of vascular smooth muscle.
71
what is the MoA of sildenafil
- Potent cGMP specific phosphodiesterase inhibitor
- As with iNO, cGMP activation results in phosphorylation which reduces calcium mobilisation within a vascular smooth muscle cell
- Phosphodiesterase breaks down cGMP therefore the inhibitor results in increased circulating levels
72
why is it important to maintain SBP in PPHN
What agents can be used to maintain BP
if low blood pressure, will worsening R --> L shunting and therefore hypoxia and acidosis
Dopamine infusion
Dobutamine infusion
Adrenaline infusion
73
What is the difference between venarterial and veno-veno ECMO
venoarterial goes from R) atrium --> Ecmo --> Aorta
-have to divide the common carotid artery
-increased risk of stroke
-lung and heart bypass
veno-veno: jugular vein --> ecmo --> RV
-lung bypass only
-nil control over cardiac output
-dont have to divide the aorta
74
What are 4 contra-indications to ECMO
< 34 weeks
<2kg
Current bleeding
Irreversible lung damage
75
What stage of lung development do diaphragmatic hernias occur
Embryonic and pseudo glandular
76
What are the two types of diaphragmatic hernias
Which is more common
o Bochdalek:95% of cases occur posterolaterally. 90% happen on the left side.
o Morgagni: Retrosternal.
77
How do you calculate the oxygenation index for ECMO
What OI is normal
What OI indicated referral for ECMO
OI = MAP X FiO2 / Pa O2
- Mean airway pressure
- PaO2 = arterial oxygen content
Index
- OI <5 = normal
- OI >10 = severe oxygenation problem
- OI >20 = extreme oxygenation problem
- OI > 40= ECMO referral
78
In an infant with no microsomal abnormality what other factor determines survival in pulmonary hypoplasia
Degree of residual functioning lung
Demonstrated by the lung volume: head size ratio
If the lung volume to head size ratio is < 1 almost no infants survive.
If the lung volume to head size ratio is > 1.4 almost all survive.
79
What are common issues in a baby with congenital diaphragmatic hernia
PPHN
Pulmonary hypoplasia
GORD- 50% affected
40% mortality rate
80
What is the pathogenesis of CPAM
What are the types
o Abnormal branching morphogenesis of the lung.
o Molecular mechanisms are unknown but may include an imbalance between cell proliferation and apoptosis during organogenesis
o Disorders of HOXB5 have been implicated in the process
Types
based on the LOCATION, plus the size of the cyst and their cellular characteristics
Type 0: trachea and proximal bronchus- small cysts – very rare- fatal
Type 1 (65%)- distal bronchi and proximal bronchioles:
distinct thin walled cysts- usually single but may be multi-loculated.
Well differentiated tissue so felt to originate late in embryogenesis.
Single or multiple large cysts.
Despite shift effects, there is usually a good prognosis
Type 2 (20%)- terminal bronchioles
multiple small cysts which blend into adjacent normal tissue.
Cysts resemble dilated terminal bronchioles.
Associated with other congenital abnormalities in 60% of cases e.g. TOF, bilateral renal agenesis
Type 3 (5-10%): near alveolus
very large and can involve entire lobe or several lobes.
Cysts develop near the alveolus and are so small they look like a solid mass
Can be a mixture of cystic and solid tissue.
Due to their size and lack of differentiation, thought to originate early in pregnancy.
Worst prognosis
Type 4: distal alveolar
81
In pulmonary sequestration when blood supplies the non functional lung
Systemic blood normally from a branch of the thoracic aorta
82
What is schmiter syndrome
R) lung hypoplasia and TAPV with drainage of the right pulmonary vein into the IVC or R) atrium
83
when does a tension pneumothorax occur
Tension pneumothorax occurs if the accumulation of air within the pleural space is sufficient to elevate intra-pleural pressure above atmospheric pressure
84
what cardiac condition is associated with pulmonary haemorrhage
PDA
85
In mechanical ventilation what factors affect
-oxygenation
-ventilation
- Oxygenation (PaO2) is determined by the Inspired oxygen fraction (FiO2) and Mean airway pressure (MAP).
- Ventilation (CO2 clearance) is determined by the Minute Volume (MV= Tidal Volume (VT) x Rate (R)).
86
when setting a volume guarantee what will the machine adjust to ensure a volume is delivered with each breath
the PIP
87
when will Volume guarantee not work
When there is a large leak around the ETT
88
what is the difference between
PC-AC
SIPPV
PC-SIMV
PC-PSV
PC-AC = pressure control- assist control
SIPPV= synchronised intermittent positive pressure ventilation
BOTH the above are the same thing by different names- each breath the baby takes is assisted by the ventilator
PC-SIMV: pressure control-synchronised intermittent mandatory ventilation = Supports a certain number of breaths- if baby breathing above this doesn't support this
Think-Mandatory = uncompromising = will only assist set breaths
PC-PSV= pressure control pressure support ventilation- similar to PC-AC; all breaths are supported BUT baby also determines the DURATION of the breath
89
In HFOV how do you improve ventilation
Increase amplitude
Decrease frequency
90
How do you calculate the ventilation index
What do the numbers mean
VI= PCO2 x RR x PIP / 1000
VI > 70 = severe respiratory failure
VI > 90 = very severe failure; consider ECMO
91
what is the insensible fluid loss in premature infants
2-3ml/kg/hr
92
What it the step increase in fluid requirements for a day 1 - day 7 baby
How many milli-mols of Na/K/Ca supplementation
ml/kg/day
60
75
90
105
120
150
180
Na=3
K= 2
Ca=1
93
What would be 3 reasons for neonatal hyperkalaemia
-haemolysis or red cell break down
-Congenital adrenal hyperplasia
-severe metabolic acidosis
94
How many kcal/day does a preterm vs term infant need
120 vs 100kcal/day
95
What is the kcal content of glucose, protein and fat
Fat= 9
Glucose=4
Protein = 4
96
why is cows milk not recommended in first year of life
low iron bioavailability
97
what is the absorbable content of iron in breast milk vs cows miilk
breast= 50%
cows=10%
98
what is the dose of prophylactic iron
who is it given to
1ml/kg/day
<37 weeks OR <2500g
until.1 year of age
99
what immunoglobulin is found in breast milk
IgA
100
what signs in Bells scoring system for NEC
stage
1
IIA
IIB
IIIA
IIIB
Stage 1- apnoea, temp instability, feed intolerance, occult blood in stool normal AXR
Stage 2A: apnoea, temp instability feed intolerance, GROSSLY BLOODY STOOL, FOCAL pneumatosis on AXR
Stage 2B: Thrombocytopenia, mild metabolic acidosis, ABDOMINAL WALL OEDEMA, palpable loops and tenderness, WIDESPREAD pneumoatosis
Stage 3A: mixed acidosis, coagulopathic, WORSENING ASCITES, NO free air, worsening wall oedema with erythema and induration
Stage 3B: Pneumoperitoneum- perforated bowel- shock and deterioration
101
what stage of NEC do you get pneumoperitoneum
Stage 3B
102
what stage of NEC do you get grossly bloody stool and focal pneumatosis
Stage IIA
103
what stage of NEC do you get abdominal wall oedema, widespread pneumoatosis and thrombocytopenia
stage IIB
104
What stage of NEC do you get
-thrombocytopenia
-coagulopathy
IIB
IIIA
105
What stage of NEC do you get worsened wall oedema with erythema plus no free air in bowel and mixed acidosis
IIIA
106
what is pneumatosis
presence of gas in the wall of the intestine
107
What % of infants with NEC have bacteraemia
20%
108
What haematology sign is associated with impending bowel perforation in NEC
DIC
109
What antibiotic regime is used in NEC
Amoxicillin- enterococcus
Gentamycin- Gram -ve
Metronidazole - anaerobes
110
Describe the management of NEC
-NBM and start TPN for 7-14 days
-IV Abx- Amox/Metronidazole/Gent
-NG gastric decompression- replace gastric losses IV
-Strict fluid balance
-Surgery if perforation OR failure of medical management
-Post surgery do not perform rectal temp
111
What area of gut resection in NEC will cause the most significant malabsorption
Jejunum- where most amino acids and disaccharides absorbed
112
what condition is meconium Ileus associated with
CF
113
What is the first line treatment in meconium ileus
Gastrograffin enema
114
Describe Type A, B, C, D and E TOF
Type A- oesophageal atresia but no fistula
Type B- oesophageal atresia with proximal TOF
Type C- oesophageal atresia with distal TOF
Type D- oesophageal atresia with proximal and distal TOF
Type E- nil atresia but a TOF
115
What is the most common type of TOF and describe it
What % have this version
Type C- oesophageal atresia with distal TOF
85%
116
How does a Type E TOF frequently present
Describe a Type E TOF
Recurrent LRTIs
No atresia but a TOF
117
What syndrome are most TOFs associated with and what does are the signs
VACTREL
-Vertebral
-Anal abnormalities
-Cardiac abnormaliteis
-Tracheal-oesophageal anomalies
-Renal anomalies
-Ears
-Limbs
118
What are some complications post atresia/TOF repair
stricture
GORD
re-fistulisation
dysphagia
118
What are some complications post atresia/TOF repair
stricture
GORD
re-fistulisation
dysphagia
119
What is the cause of duodenal atresia
What syndrome is it associated with
Intestinal tract is blocked by endoderm epithelium
Downs syndrome
120
What is the presentation of duodenal atresia
Vomitting without abdominal distension in the first day of life
121
At what week does the midgut return to the abdomen in utero
10-11
122
What is the centre of intestinal rotation
the superior mesenteric artery
123
Where should the duodenal junction and caecum be relative to the spine in a correctly rotated bowel
cacum- RLQ
DJ- LUQ
124
What condition is a LADD procedure used for and what does it involve
Malrotation
Division of the LADD bands of peritoneum, correction of Volvulus, appendectomy and functional positioning +/- Internal fixation
125
What is a persistent cloaca
Failure to divide the cloaca into a separate return and Genitourinary system - rectum, vagina and urinary tract are joint be a single channel
126
What causes an mechkles diverticulum
incomplete obliteration of the vitilline duct
126
What causes an mechkles diverticulum
incomplete obliteration of the vitilline duct
127
what is the rule of 2s in a meckles diverticulum
2% of population
2 inches in length
2 feet from ileocaecal valve
2 types of tissue- gastric and pancreatic
Presents before age of 2
128
what 2 types of tissue are contained in the ileocaecal valve
pancreatic
gastric
129
What test is used to diagnose meckles
Technitium-99
Taken up by gastric mucosa = stomach and meckles
130
what is the difference in pathogenesis between gastroschisis and omphalmocele
gastroschisis- failure of anterior abdominal wall closure
omphalocele- failure of midgut to return to abdominal cavity during development
131
what % of omphalmocele are associated with other congenital abnormalities
75%
132
what are the signs of Hirschsprung's disease
bowel obstruction relieved with faecal examination
133
what procedure is contraindicated in hypospadius
circumcision as preputial skin often used to reconstruct the urethra
134
what is the difference between
-spina bifida occulta
-meningocele
-myelomengiocele
SPO: defect in vertebral closure but no protrusion of meninges or spinal cord
Meningocele: protrusion of meninges with cyst formation but no protrusion the spinal cord
Myelomeningocele: herniation of the meninges, nerve roots and spinal cord though the dorsal vertebral defect
135
In which periventricular area doe premature infants experience IVH
subependymal germinal matrix
136
at what gestation is there involution of the germinal matrix
34 weeks gestation
137
what are the indications for a cerebral ultrasound
<32 weeks gestation OR <1250g
Clinical suspicion
Monitoring of hydrocephalus
MCDA twins
138
Describes the 4 grades of IVH
Grade 1- germinal matrix
Grade 2- IVH but no ventricular dilitation
Grade 3- IVH with ventricular dilatation
Grade 4- intra-parenchymal haemorrhage that is localised or extensive (involving 2+ regions)
139
what is the most common adverse outcome associated with IVH
Cerebral palsy
140
What is the % affected by cerebral palsy in
PIVH requiring shunt
Grade 4 IVH
Grade 3 IVH
75%
89%
50%
141
what type of cerebral palsy does PVL result in
spastic diplegia
142
what happens in primary vs secondary neuronal injury in HIE
primary injury- hypoxia results in anaerobic respiration- resulting in accumulation of cytotoxic metabolites e.g. lactate and glutamate AND fails to produce enough ATP for Na/K ATPase so sodium accumulates causing cell damage --> cell death and cerebral oedema
secondary injury is a reperfusion injury - hypo perfusion then reperfusion with resuscitation results in neutrophil activation and the release of free radicals and prostaglandin --> apoptosis and death
143
what are signs of severe encephalopathy on a sarnat score
absent - only responds to painful stimuli
spontaneous movement - none
flaccid tone
absent suck
absent moro
apnoea
144
what are sarnat signs of moderate encephalopathy
reduced responsive to non-painful stimuli
reduced spontaneous activity
hypotonia
incomplete moro
incomplete suck
periodic breathing
145
What weight, gestational age and severity of HIE allows for hypothermia
Weight >1800g
Gestation age >35 weeks
Severity: moderate-severe
146
what Apgar score at 10 minutes under which do we consider therapeutic cooling
During what window must it be initiated
<5 at 10 minutes
6 hours
147
how long is therapeutic cooling for in HIE
72 hours
148
what is the first line seizure treatment in neonatal HIE
Phenobarbital
149
what fuses in
-brachiocephaly
-scaphocephaly
-plagiocephaly
-trigonocephaly
-brachycephaly= short head = coronal suture
-sachpocephaly = 'boat head' = saggital suture
-plagiocephaly = assumetrical lambed or coronal suture
trigonocephaly- metopic suture
150
what are the three zones in the retina
what are the stages of ROP
Zone 1: optic nerve - macula center - 2x this as a radius
Zone 2: radius from the optic nerve to the nasal ora serrate
Zone 3: remainder of outer boarder of zone 2 to the temporal serrate
stage 1- demarcation line
stage 2- ridge
stage 3- extra retinal fibrovascular proliferation
stage 4- partial retinal detachment
stage 5- total retinal detachment
151
what is plus disease
tortuous retinal vessels
retinal haze
pupil rigidity
152
why does ROP occur
oxygen toxic to premature eyes
results in retinal vasoconstriction
if vasoconstriction occurs for long enough will get obliteration
then will get neovascularisation
153
who is screened for ROP
<30 weeks
<1250g
plus consider:
Twin-twin transfusion
prolonged NO for PPHN
severe sepsis
Grade 3 or 4 IVH
154
when does zone 1 ROP get treated
Zone 1 AND plus
OR
Zone 1 stage 3
155
when is zone 2 ROP treated
stage 2-3 AND plus
