Neonatology Flashcards
1
Q
What do TY2 alveolar cells produce surfactant?
A
24-34 weeks gestation
2
Q
Purpose of surfactant
A
Redcued surface tension in alveoli and prevents them from collapsing
Maximises surface area
Increased lung compliance
Promotes equal expansion of all alveoli
3
Q
Foramen ovale-> fossa oval is
A
First breath Alveoli expands Decreased pulmonary vascular resistance Fall in right atrial pressure L>R Squashes atrial septum Functional closure Fossa ovalis
4
Q
Ductus arteriosus-> ligamentum arteriosum
A
Increased blood oxygenation with first breath
Drop in circulating prostaglandins
Closure of ductus arteriosus
Ligamentum arteriosum
5
Q
Ductus venosum-> ligamentum venosum
A
Umbilical cord is clamped
Reduced blood flow in umbilical veins
6
Q
Hypoxia in neonate
A
Normal in labour and birth
Placenta can’t carry out normal gas exchange during contractions
Extended hypoxia: anaerobic respiration and drop in fetal heart rate
Reduced consciousness and drop in respiratory effort
Hypoxic-ischaemic encephalopathy
Cerebral palsy
7
Q
Issues in neonatal resuscitation
A
Hypoxia
Large surface area to weight ratio, get cold very easily
Born wet, so lose heat rapidly
May have meconium in mouth or airway
8
Q
Principles of neonatal resuscitation
A
Warm baby
Calculate APGAR score
Stimulate breathing, neutral position, towel dry, check for meconium
Inflation breaths: 2 cycles of 5, give oxygen if pre-term
Chest compressions: 3:1 with ventilation, if <60bpm
9
Q
APGAR score
A
Appearance (skin colour) Pulse Grimace (response to stimulation) Activity (muscle tone) Respiration
10
Q
Delayed umbilical cord clamping
A
Placental transfusion: Fetal blood in placenta enters circulation of baby
Improved Hb, iron stores, blood pressure
Reduction in intraventricular haemorrhage and necrotising enterocolitis
Increase in neonatal jaundice, requiring more phototherapy
11
Q
Care immediately after birth
A
Skin to skin Clamp the umbilical cord Dry the baby Keep warm VitaminK Label the baby Measure the weight and length
12
Q
Vitamin K after birth
A
Babies born with deficiency
IM injection
Stimulate cry- expand lungs
Helps prevent bleeding: intracranial, umbilical stump, GI bleeding
Oral takes longer to act, doses at birth, 7 days, 6 weeks
13
Q
Skin to skin contact after birth
A
Helps warm baby
Improves interaction
Calms baby
Improves breast feeding
14
Q
Care after delivery room
A
Initiate breast feeding or bottle feeding as soon as baby is alert enough
Newborn examination within 72 hours
Blood spot test
Newborn hearing test
15
Q
Blood spot screening
A
Day 5 Sickle cell disease Cystic fibrosis Congenital hypothyroidism Phenylketonuria Medium-chain acyl-COA deydrogenase deficiency Maple syrup urine disease Isovaleric acidaemia Glutaric aciduria TY1 Homocystin
16
Q
Newborn examination
A
Performed within 72hours after birth Repeated after 6-8weeks Ask if baby has passed meconium? Is the baby feeding ok? FH of congenital problems
17
Q
Oxygen saturations pre-ductal or post-ductal
A
Before and after ductus arteriosus
No more than 2% difference
Pre-ductal: right hand
Post-ductal: either foot
18
Q
When does the ductus arteriosus close?
A
1-3 days
19
Q
General appearance newborn examination
A
Colour (pink is good)
Tone
Cry
20
Q
Head examination neonates
A
General appearance: size, shape, dysmorphology, caput succedaneum, cephalohaematoma, facial injury
Occipital frontal circumference
Fontanelles
Sutures
Ears
Eyes: squint, epicanthic folds, purulent discharge
Red reflex: congenital cataracts and retinoblastoma
Mouth: cleft lip or tongue tie
Suck reflex
21
Q
Shoulder and arm examination neonates
A
Shoulder symmetry: clavicle fracture Arm movements: Erb’s palsy Brachial pulses Radial pulses Palmar creases: Down’s Digits: clinodactyly Sats probe: preductal reading on right wrist
22
Q
Chest newborn examination
A
Oxygen sats in right wrist and foot
Observe breathing: resp distress, symmetry, stridor
Heart sounds: murmurs, heart sounds, HR, identify which side heart is on
Breath sounds: symmetry, good air entry, added sounds
23
Q
Abdomen examination newborn
A
Observe shape: diaphragmatic hernia
Umbilical stump: discharge, infection, periumbilical hernia
Palpate for organometallic, hernias or masses
24
Q
Genitals examination in newborn
A
Observe for sex, ambiguity, obvious abnormalities
Palpate testes and scrotum: check both are present and descended, check for hernias or hydrocele
Inspect the penis for hypospadias, epispadias, urination
Inspect anus for patency
Ask about meconium and if baby has opened the bowel
25
Legs examination newborn
Observe the legs and hips for equal movements, skin creases, tone and talipes
Barlow and Ortolani movements; check for clunking, clicking and dislocation of the hips
Count the toes
26
Back examination newborn
Inspect and palpate spin
Curvature, spina bifida, pilonidal sinus
Sacral dimples/pits for tethered spine
27
Reflexes in newborn
```
Moro reflex
Suckling reflex
Rooting reflex: tickling cheek
Grasp reflex
Stepping reflex
```
28
Skin findings in neonatal examination
```
Haemangiomas
Port wine stains
Mongolian blue spot
Cradle cap
Desquamation
Erythema toxicum
Milia
Acne
Naevus simplex
Moles
Transient pustular Melanosis
```
29
Talipes
Clubfoot
Ankles in supinated position, rolled inwards
Positional vs structural
30
Positional talipes
Muscles slightly tight around ankle
Bones unaffected
Physiotherapy referral
31
Structural talipes
Involves bones of foot and ankle
| Requires referral to orthopaedic surgeon
32
Undescended testes
Require monitoring and referral to a urologist
33
Haemangiomas
If near eyes, mouth, or affecting airway
Propanolol
If not, monitor
34
Port wine stains
Capillary abnormalities
Pink patches of skin on face
Dont fade, turn a darker red or purple
Can be relate to Sturge-Weber syndrome: visual impairment, learning difficulties, headaches, glaucoma, epilepsy
35
Sturge-Weber syndrome
```
Port wine stains
Visual impairment
Learning difficulties
Headaches
Epilepsy
Glaucoma
```
36
Clunky or asymmetrical hips
Require referral for hip US
| To rule out DDH
37
Cephalohaematomas
Require monitoring for jaundice and anaemia
38
Soft systolic murmur
If grade 2 or less
Health neonate
Resolve after 24-48, patent foramen ovale closes shortly after birth
39
Caput succedaneum
Oedema on scalp, outside periosteum, so can cross sutures lines
Cause: pressure on scalp during delivery
Self resolves in a few days
40
Cephalohaematoma
```
Collection of blood between skull and periosteum
Traumatic subperiosteal haematoma
Doesn’t cross suture lines
Self-resolves in a few months
Risk of anaemia and jaundice
```
41
Facial paralysis
Forceps delivery
Function resolves within a few months
Neurosurgical input may be required when function doesn’t return
42
Erb’s palsy
```
Injury to C5/6
Shoulder dystocia, traumatic or in instrumental delivery and large birth weight
Internally rotated shoulder
Extended elbow
Flexed wrist facing backwards (pronated)
Lack of movement in affected arm
Function returns within a few months
```
43
Fractured clavicle
Shoulder dystocia, traumatic or instrumental delivery and large birth weight
Noticeable lack of movements or asymmetry of movement in the affected arm
Asymmetry of the shoulders, with the affected shoulder lower than the normal shoulder
Pain and distress on movement of the arm
USS/XRAY
Conservative mx
Potential brachial plexus injury
44
Common organisms in neonatal sepsis
```
Group B streptococcus (vagina)
E.coli
Listeria
Klebsiella
Staph. Aureus
```
45
Risk factors
```
Vaginal GBS colonisation
GBS sepsis in a previous baby
Maternal sepsis, chorioamnionitis or fever >38
Prematurity, <37weeks
Early rupture of membrane
Prolonged rupture of membranes
```
46
Clinical features of neonatal sepsis
```
Fever
Reduced tone and activity
Poor feeding
Respiratory distress or apnoea
Vomiting
Tachycardia or bradycardia
Hypoxia
Jaundice within 24hours
Seizures
Hypoglycaemia
```
47
Red flags for neonatal sepsis
Confirmed or suspected sepsis in mother
Signs of shock
Seizures
Term baby needing mechanical ventilation
Respiratory distress starting >4 hours after birth
Presumed sepsis in another baby in a multiple pregnancy
48
Treatment of presumed sepsis
If >1 risk factor or clinical features, monitor observations and clinical condition for at least 12hours
If >2 risk factors or clinical features of neonatal sepsis, start ax
If single red flag give ax within 1 hr, after blood cultures
FBC, CRP
Lumbar puncture if features of meningitis
49
Ax for neonatal sepsis
Benzylpenicillin and gentamicin
| If lower risk can give cefotaxime
50
Ongoing mx of neonatal sepsis
Check CRP at 24 hours , <10
Blood culture at 36hours, negative
Stop ax
If >10 lumbar puncture needed
Check CRP after 5 days if still on treatment, if lumbar puncture, blood culture and CRP negative and clinically well, stop ax
51
What is prematurity
32-37 weeks gestation: moderate to late preterm
28-32: very preterm
<28 extreme preterm
52
Associations with prematurity
```
Social deprivations
Smoking
Alcohol
Drugs
Overweight or underweight mother
Maternal co-morbidities
Twins
Personal or FH of prematurity
```
53
Management of prematurity before birth
| Women with a history of pre-term birth or US demonstrating a cervical length of 25mm or less before 24 weeks gestation
Prophylactic vaginal progesterone
Prophylactic cervical cerclage: suture to close cervix
Tocolysis with nifedipine
Maternal corticosteroids: befor 35weeks, reduce neonatal mortality
IV Mgsulphate: protect baby’s brain, given before 34weeks
Delayed cord clamping or cord milking: increased circulating volume and Hb
54
Issues in early life of premature babies
```
RDS
Hypothermia
Hypoglycaemia
Poor feeding
Apnoea and bradycardia
Neonatal jaundice
Intraventricular haemorrhage
Retinopathy of prematurity
Necrotising enterocolitis
Immature immune system and infection
```
55
When to suspect hypoxic-ischaemic encephalopathy
Events that could lead to hypoxia during the perinatal or intrapartum period
Acidosis on umbilical artey blood gas
Poor APGAR score
Evidence of multi organ failure
56
Causes of hypoxic ischaemic encephalopathy
```
Anything that leads to brain asphyxia
Maternal shock
Intrapartum haemorrhage
Prolapsed cord
Nuchal cord
```
57
What is the staging for HIE?
Sarnat staging
58
Mild HIE
Poor feeding, generally irritability and hyper-alert
Resolves within24hrs
Normal prognosis
59
Moderate HIE
Poor feeding, lethargic, hypotonic and seizures
Can take weeks to resolve
Up to 40% develop cerebral palsy
60
Severe HIE
Reduced consciousness, apnoea, flaccid and reduced/absent reflexes
50% mortality
90% develop cerebral palsy
61
Therapeutic hypothermia
HIE mx
Neonatal ICU: cooling blanket and cooling hat
33-34 target, use rectal probe for 72hours
Baby is gradually warmed to normal temperature after 6 hours
62
Intention of therapeutic hypothermia
Inflammation and neurone loss
Acute hypoxic injury
Reduces risk of cerebral palsy, developmental delay, learning disability, blindness and death
63
Physiological neonatal jaundice
Fetal RBC break down more rapidly
Bilirubin excreted via placenta, at birth rise in bilirubin
Mild yellowing of skin and sclera at 2-7days of age
Usually resolves completely by 10 days
64
Causes of increased production of bilirubin
```
HDN
ABO incompatibility
Haemorrhage
Intraventricular haemorrhage
Cephalohaematoma
Polycythemia
Sepsis and DIC
G6PD deficiency
```
65
Depressed clearance of bilirubin
```
Prematurity
Breast milk jaundice
Neonatal cholestasis
Extrahepatic biliary atresia
Endocrine disorders (hypothyroid and hypopituitary)
Gilbert syndrome
```
66
Jaundice within 24 hours of birth
Treatment for sepsis
| Pathological
67
Jaundice in premature neonates
Immature liver
| Increases complications, kernicterus (brain damage)
68
Breast milk jaundice
Components of breast milk inhibit ability of liver to process bilirubin
Inadequate breastfeeding: slow passage of stool (dehydration), increased absorption of bilirubin in intestines
Encourage breastfeeding though
69
HDN
Cause of haemolysis and jaundice in neonate
Rh-ve mother makes antibodies against Rh+ve baby
Haemolysis
Anaemia
High bilirubin
70
Prolonged jaundice
>14 days in full term babies
>21 days in premature babies
Check for biliary atresia, hypothyroidism, G6PD deficiency
71
Investigations for neonatal jaundice
FBC and blood film; polycythemia or anaemia
Conjugated bilirubin: biliary atresia
Blood type testing
Direct Coombs test for haemolysis
Thyroid function tests
Blood and urine cultures if infection is suspected
G6PD deficiency
72
Phototherapy for neonatal jaundice
Converts unconjugated bilirubin into isomers that can be excreted into bile and urine without require it conjugation in the liver
Eye-patches to protect eyes
Light-box shines UV light on baby’s skin
Double phototherapy involves two light boxes
Bilirubin is closely monitored during treatment
Rebound bilirubin measured 12-18hours after stopping
73
Kernicterus
Bilirubin crosses blood brain barrier
Direct damage to CNS
Cerebral palsy, learning disability, deafness
74
Apnoea in neonates
Breathing stops for >20 seconds
Oxygen desaturation or bradycardia
Accompanied by a period of bradycardia
Very common in premature neonates, <28 weeks gestation
75
Causes of apnoea of prematurity
```
Immaturity of ANS that controls respiration and HR
Infection
Anaemia
Airway obstruction
CNS pathology, seizures or headaches
GORD
Neonatal abstinence syndrome
```
76
Management of apnoea of prematurity
Apnoea monitors
Tactile stimulation
IV caffeine
Episodes will settle as baby grows and develops
77
Retinopathy of prematurity
Mostly affects babies born before 32weeks gestation
Abnormal development of retinal blood vessels
Scarring, retinal detachment, blindness
Treatment can prevent blindness
78
Pathophysiology of retinopathy of prematurity
Retinal blood vessel development: 16weeks-40weeks
Stimulated by hypoxia
In pre-term, less hypoxia exposure so stimulation removed
Hypoxic environment returns: excessive blood vessels (neovascularisation) and scar tissue
Abnormal blood vessels regress
Scar tissue causes retinal detachment
79
Assessment of retinopathy of prematurity
Stage 1: slightly abnormal vessel growth
Stage 5: complete retinal detachment
Plus disease: additional findings, tortuous vessels, hazy vitreous humour
80
Retina divided into three zones
Zone 1: optic nerve and macula
Zone 2: edge of zone 1 to ora serrata (pigmented border between retina and ciliary body)
Zone 3: outside ora serrata
81
Screening for retinopathy of prematurity
Babies born before 32 weeks or <1.5kg
At 30-31 weeks gestational age in babies born before 27weeks
4-5 weeks of age in babies born after 27weeks
Screening every 2 weeks until vessels reach zone 3
82
Treatment of retinopathy of prematurity
First line: transpupillary laser photo coagulation and reverse neovascularisation
Cryotherapy
Injections of intravitreal VEGF inhibitors
Surgery if retinal detachment occurs
83
Respiratory distress syndrome
Affects premature neonates
Those born before lungs start producing adequate surfactant
Occurs below 32weeks
CXR shows ground glass appearance
84
Pathophysiology of respiratory distress syndrome
```
Less surfactant
High surface tension in alveoli
Atelectasis
Cant expand
Inadequate gas exchange
Hypoxia, hypercapnia, respiratory distress
```
85
Management of respiratory distress syndrome
Antenatal steroids (i.e. dexamethasone)
Increase surfactant production
Reduce incidence and severity
86
Management of respiratory distress syndrome in premature neonates
Intubation and ventilation to assist breathing
Endotracheal surfactant
CPAP
Supplementary oxygen 91-95%
87
Short term complications of RDS
```
Pneumothorax
Infection
Apnoea
Intraventricular haemorrhage
Pulmonary haemorrhage
Necrotising enterocolitis
```
88
Long-term complications of RDS
Chronic lung disease of prematurity
Retinopathy of prematurity
Neurological, hearing and visual impairment
89
Risk factors for necrotising enterocolitis
```
Very low birth weight or very premature
Formula feeds
Respiratory distress and assisted ventilation
Sepsis
PDA or congenital heart defects
```
90
Presentation of necrotising enterocolitis
```
Intolerance to feeds
Vomiting, green bile
Generally unwell
Distended, tender abdomen
Absent bowel sounds
Blood in stools
If perforated: signs of shock
```
91
Investigations for necrotising enterocolitis
Bloods: FBC, CRP, capillary blood gas, blood culture
AXR: supine position, lateral, ap, lateral decubitus
92
AXR of necrotising enterocolitis
```
Dilated loops of bowel
Bowel wall oedema (thickened bowel walls)
Pneumatosis intestinalis
Pneumoperitoneum: indicates perforation
Gas in portal veins
```
93
Management of necrotising enterocolitis
```
Nil by mouth
IV fluids
TPN
NG tube
Surgical team
Surgery to remove dead bowel tissue
Temporary stoma if significant bowel is removed
```
94
Complications of necrotising enterocolitis
```
Perforation and peritonitis
Sepsis
Death
Strictures
Abscess formation
Recurrence
Long-term stoma
Short bowel syndrome after surgery
```
95
Substances that can cause neonatal abstinence syndrome
```
Opiates
Methadone
Benzodiazepines
Cocaine
Amphetamines
Nicotine or cannabis
Alcohol
SSRI antidepressants
```
96
When do withdrawal symptoms occur?
24hrs-21days:
Methadone
Benzodiazepines
```
3-72hours after birth:
Opiates
Diazepam
SSRI
Alcohol
```
97
CNS signs and symptoms of neonatal abstinence syndrome
```
Irritability
Increased tone
High-pitched cry
Not settling
Tremors
Seizures
```
98
Vasomotor and resp signs and symptoms of neonatal abstinence syndrome
Yawning
Sweating
Unstable temperature and pyrexia
Tachypnoea (fast breathing)
99
Metabolic and GI signs and symptoms of neonatal abstinence syndrome
Poor feeding
Regurgitation or vomiting
Hypoglycaemia
Loose stools with a sore nappy area
100
Management of neonatal abstinence syndrome
Monitoring on NAS chart for at least 3 days
Urine sample from neonate
Oral morphine sulphate for opiate withdrawal
Oral phenobarbitone for non-opiate withdrawal
101
Management of neonatal abstinence syndrome
Test for Hep B/C and HIV
Safeguarding and social service involvement
Safety-net advice for readmission if withdrawal signs and symptoms occur
Follow-up from paediatrics, social services, health visitors and the GP
Support for the mother to stop using substances
Check suitability for breastfeeding
102
Effects of alcohol in early pregnancy
```
<3 months of pregnancy
Miscarriage
Small for dates
Pre-term delivery
Fetal alcohol syndrome
```
103
Fetal alcohol syndrome characteristics
```
Microcephaly
Thin upper lip
Smooth flat philthrum
Short palpable fissure
Learning disability
Behavioural difficulties
Hearing and vision problems
Cerebral palsy
```
104
Congenital rubella syndrome
Mother becomes infected during pregnancy
MMR vaccine for women thinking about pregnancy
Pregnancy women shouldn’t be given vaccine
105
Features of congenital rubella syndrome
Congenital cataracts
Congenital heart disease (PDA and pulmonary stenosis)
Learning disability
Hearing loss
106
Chickenpox in pregnancy
Fetal varicella syndrome
Severe neonatal varicella infecton if mum infected around delivery
Varicella pneumonitis, hepatitis, encephalitis
Give vaccine before or after pregnancy
107
Exposure to chickenpox in pregnancy
Safe if previously had chickenpox
Test VZV IgG levels, if positive they’re safe
If not immune, give IV varicella Ig as prophylaxis, treat within 10 days of exposure
When rash starts to develop, treat with oral aciclovir if present within 24hrs and are <20 weeks gestation
108
Features of congenital varicella syndrome
Infection within 28 weeks of gestation
Fetal growth restriction
Microcephaly, hydrocephalus and learning disability
Scars and significant skin changes following dermatomes
Limb Hypoplasia
Cataracts and eye inflammation (chorioretinitis)
109
Features of congenital CMV
```
Fetal growth restriction
Microcephaly
Hearing loss
Vision loss
Learning disability
Seizures
```
110
Congenital toxoplasmosis
Intracranial calcification
Hydrocephalus
Chorioretinitis
111
Congenital Anika syndrome features
Microcephaly
Fetal growth restriction
Other intracranial abnormalities: ventriculomegaly, cerebellar atrophy
Pregnant women in contact should be tested with viral PCR and antibodies to Zika virus
112
Risk factors for sudden infant death syndrome
Prematurity
Low birth weight
Smoking during pregnancy
Male baby (only slight increased risk)
113
Minimising the risk of sudden infant death syndrome
Put baby on back
Keep head uncovered
Place their feet at foot of bed to prevent sliding Godwin
Keep cot clear
Maintain comfortable room temperature
Avoid smoking and handling baby after smoking
Avoid co-sleeping, particularly on sofa or chair
Lullaby trust
Care of next infant team
114
Definition of neonate
<4 weeks
115
Very low birth weight:
| Extremely low birth weight:
Very low birth weight: <2.5kg
| Extremely low birth weight: <50kg
116
Congenital diaphragmatic hernia
Incomplete formation of diaphgram
Abdominal viscera herniate
Stops lungs from inflating: pulmonary Hypoplasia and HTN
Respiratory distress after birth
Use anaesthetic drugs to stop breathing
Secure airway
117
Management of pre-term baby
Ventilation
NG tube: unable to coordinate suck and swallow due to immature brain
TPN, central line (umbilical)
Incubator: heats and humidifies air
Convection, lying in-utero position to protect joints
118
Risk factors for hip problems
Breech baby
Connective tissue disorder
Talipes/ club foot
119
Hearing screen
Otoacoustic emissions
| Automated auditory brainstem response
120
Capillary haemangioma
Strawberry naevus
Grows then involuntes
Can be treated with propanolol
121
Toxic erythema of the newborn
Rash
Begins on face and spreads to affect trunk and limbs
Palms and soles usually not affected
Waxes and wanes over several days
122
Positional talipes
No medical intervention needed
| Foot rests down and inwards
123
Minor breastfeeding problems
Frequent feeding
Nipple pain: poor latch
Blocked duct (milk bleb): nipple pain when breastfeeding
Nipple candidiasis
124
Management of blocked duct breastfeeding
Continue breastfeeding
Positioning advice
Breast massage
125
Treatment for nipple candidiasis
Miconazole cream for mum
| Nystatin suspension for baby
126
When to treat mastitis
Systematically unwell
Nipple fissure present
Symptoms don’t improve after 12-24hrs of effective milk removal
Culture indicates infection
127
Management of mastitis
Flucloxacillin 10-14days
Continue breastfeeding or expressing
If it develops into breast abscess: incision and drainage
128
Features of breast engorgement
Occurs in first few days after infant is born
Almost always affects both breasts
Pain/discomfort typically worse just before feed
Poor milk flow
Infant may find it difficult to attach and suckle
Fever settles within 24hrs
Red breast
129
Management of breast engorgement
Hand expression of milk
130
Complications of breast engorgement
Blocked milk ducts
Mastitis
Difficulties with breastfeeding and milk supply
131
Features of Raynaud’s disease of the nipple
Pain is intermittent and present during and immediately after feeding
Blanching of nipple may be followed by cyanosis and/or erythema
Nipple pain resolves when nipples return to normal colour
132
Management of Raynaud’s disease of nipple
Minimising exposure to cold
Heat packs following breastfeed
Avoid caffeine
Stop smoking
Oral nifedipine
133
Drug contraindications to breastfeeding
```
Antibiotics: ciprofloxacin, tetracycline, chloramphenicol, sulphonamides
Psychiatric drugs: lithium, benzodiazepines
Aspirin
Carbimazole
Methotrexate
Sulphonylureas
Cytotoxic drugs
Amiodarone
```
134
Contraindications of breastfeeding
Galactosaemia
Drugs
Viral infections
135
TORCH infections
```
Toxoplasma Gondii
Other: VZV, Parvovirus b19, listeriosis
Rubella
CMV
Herpes/ HIV
Syphilis
```
136
Oesophageal atresia
Associated with tracheo-oesophageal fistula and poly hydraminos
May6 present with choking and chaotic spells following aspiration
VACTERL associations
