Neonatology, Prematurity,

and SIDS

April 2003

Dr. Kevin Levere

Preceptor: Dr. Jeff Plant

Objectives

• An overview of common complaints seen in the ED during the neonatal period• Fever, resuscitation covered previously

• A summary of issues of prematurity that affect the ED physician

• A review of SIDS and related issues• Apnea• ALTE• Home monitoring

What is a Neonate?

• Birth to 28 days old (or one month)

• Typical vitals for a neonate born at term• HR 85-205• RR 30-60• BP systolic (5th%ile) 60

• Term: >37, <42 weeks GA

Fetal to Neonatal Transition

• Umbilical ligation initiates dramatic change• Initial respiration

• Triggered by hypoxia, acidosis, hypercarbia, external stimuli• PVR falls as lungs expand, PaO2 rises and PaCO2 falls

• SVR increases with loss of the low pressure umbilicus• PFO pressed closed; fused closed after months

• PDA (shunted 90% of flow from lungs) functionally closes within the first 24hrs; fibroses within weeks

• Response to rising PaO2 and falling PaCO2

• Cardiovascular adaptation takes months

Transition continued

• Rapid fluid shifts, up to 30ml/kg• Particularly absorbed from lung airspace

• Weight falls up to 10% from birth• Regains birth weight by 7-10 days

• All these transitions occur more slowly and with more difficulty in premature infants

Organ immaturity

• CNS• Poor thermoregulation, immature brainstem function,

incomplete myelination

• CVS• Relatively few contractile elements, therefore cardiac

output especially rate dependent

• Pulmonary• Ongoing alveolar multiplication (to school age) and

interstitial development, very compliant chest• Can double adult O2 needs for weight – shorter

interval to desaturation

Organ immaturity continued

• GI• Immature gut motility, liver (drug metabolism); low

nutrient stores (glycogen, fat)

• GU• Immature renal function (drug metabolism), poor

concentrating effect

• Hematology• Immunologic immaturity; physiologic anemia typically

follows neonatal period

• Skin• Large SA, thin, lacking subcutaneous depth

What is a Preemie?

• Born at <37 weeks GA

• Not necessarily IUGR/SGA• LBW < 2500 gm• VLBW < 1500 gm• ELBW < 1000 gm

• Prematurity and IUGR both increase neonatal morbidity and mortality

Causes of Premature Delivery

• Fetal• Distress, multiple gestation, congenital anomalies,

hydrops fetalis

• Placental• Previa, abruption

• Maternal• Preeclampsia, medical illness, infection, drug use,

uterine anomalies

• Other• PROM, iatrogenic, trauma, polyhydramnios

Prematurity

• More extreme organ immaturity• Exposes preemies to specific problems• Also similar problems as other neonates

• Increased severity or risk• Even more indistinct presentation

• Increased incidence of congenital anomalies

Prematurity to the ED MD

• By 36 weeks GA• Typically develop adequate suck-swallow

ability to “feed and grow” at home• The majority have outgrown apnea of

prematurity• Thermoregulation is adequate to handle

ambient temperatures

Prematurity to the ED MD

• Significance• They might be discharged• YOU might be the next MD to see them

Issues down the road

• ICH, PVL, increased HIE• CP, seizures, developmental delay, hydrocephalus

• CLD, hypoplasia• Reduced pulmonary reserves, more hypoxia, FTT

• Persistent Fetal Circulation• Hypoxic-ischemic insults, FTT

• GI incoordination, increased NEC• Strictures, malabsorption, FTT

• Increased incidence of SIDS

Chronic Lung Disease

• Formerly described as BPD• Defined by O2 required after 36 weeks GA• Result of RDS (HMD)

• Due to surfactant deficiency

• Complications of HMD• Mortality

• Much reduced with surfactant• Iatrogenic subglottic stenosis• PFC – hypoxia and acidosis maintain PDA • CLD – mostly in ventilated and oxygenated infants

• Incidence not changed by surfactant• Nephrolithiasis – sequela of diuretics and TPN

More on CLD

• Airway obstruction, hyperactivity and hyperinflation may be demonstrated into adolescence• Preterm infants who do not have BPD are likely to

have pulmonary function at school age that is similar to that of healthy term children

• Preterm infants who have BPD are significantly more likely to have abnormal pulmonary function at 7 years of age

• Gross SJ, et al. Effect of preterm birth on pulmonary function at school age: a prospective controlled study. J Pediatr 1998

A bit on PFC

• Ongoing R-L shunting via PFO and PDA• Due to PPHN

• Results in cyanosis, respiratory distress• Causes

• Asphyxia, meconium aspiration, sepsis, HMD, hypoglycemia, polycythemia, pulmonary hypoplasia

• Often idiopathic

• Therapy• O2, correct pH, permissive mild hypercapnia; inotropes, NO;

ECMO (needed in 5-10%)

• Prognosis• Related to response of PPHN or associated HIE

Delivery Problems

• Meconium aspiration• Residual lung problems are rare but include

symptomatic cough, wheezing, and persistent hyperinflation for up to 5-10 yr

• Prognosis depends on the extent of CNS injury from asphyxia and the presence of associated problems such as pulmonary hypertension

Delivery Trauma

• Caput succedaneum• Scalp edema, crosses sutures

• Cephalohematoma• Subgaleal hematoma

• Fracture of clavicle• Peripheral nerve injuries

• C5-6 = Erb-Duchenne paralysis• C7-8 = Klumpke paralysis

• Prognosis depends on whether neurapraxia or neurotmesis• Facial nerve palsy - hemifacial

• DDx central injury (lower 2/3 of face affected) vs agenesis of facial nucleus (Mobius syndrome) – bilateral effect

Millions in Pearls

Pass the Clearasil

Pustulence

Red Herring

Spot on

Skin problems of no concern

• Milia• Tiny keratin collections, midline palatal occurrences called

Epstein’s pearls

• Baby acne• Acne, care of maternal hormones

• Pustular melanosis• Present at birth, sterile granulocytic collections that slough,

leaving hyperpigmented base

• Erythema toxicum• Idiopathic onset day 2-3, eosinophilic collections on a red base,

fade over a week

• Mongolian spot• Benign patch present from birth, fades over years

Hyper Billy

• Alert 5 day old boy• Jaundiced from 3rd day of life

• Greedy breastfeeding to date• No perinatal risk factors for infection• Family Hx negative• Normal cardiopulmonary exam• Normal fontanelle and tone, symmetric Moro,

rooting

• Do you call this an emergency?

Hyperbilirubinemia

• Jaundice (aka icterus)• In neonates at 80-150 micromol/L (60%)

• Occurs at low end in preemies, rises slower, lasts longer

• Unconjugated bilirubin• Lipid soluble; unbound crosses BBB• Kernicterus – level of risk not strictly known

• Conjugated bilirubin• Unbound is renally excreted• Increased if >20% total bilirubin

Approach to Neonatal Jaundice

Unconjugated Hyperbilirubinemia

• Hemolytic disease• Sepsis, UTI• Hereditary or acquired

• Decreased hepatic conjugation• Decreased hepatic intake

• Breast milk, hypothyroidism

• Decreased hepatocellular function• Hepatitis• Physiologic, Crigler-Najjar, Gilbert

• Enterohepatic recirculation

Phototherapy

• Address exacerbating causes

• Empiric levels for phototherapy vs exchange transfusion based on risk of kernicterus• Early signs

• Lethargy, hypotonia, irritability

• Later signs• Posturing, hypertonicity, seizures

Conjugated Hyperbilirubinemia

• Biliary atresia• Commonest cause of liver failure in pediatrics

• CF• Bile/mucous plug ("inspissated bile")

• Management• Disease specific• No response to phototherapy or exchange transfusion

Early Anemia (first few days)

• RBC destruction• Hemolytic

• Immune – erythroblastosis fetalis, TORCHS

• RBC loss• Transplactental

• Hemorrhage vs transfusion

• Hemorrhagic disease – “early” or “classic” < 1 week• Vitamin K deficiency, intrapartum anticoagulant and

antiepileptic drug use

• IVH, liver laceration

Later Anemia

• Physiologic• Nadir at 8-12 weeks

• RBC destruction• Hemolytic

• Immune• Congenital (RBC membrane or enzyme anomalies, Hgb)

• RBC loss• Iatrogenic

• RBC depressed production – rare• Diamond-Blackfan etc.

Polycythemia

• Hematocrit > 65%• Placental transfusion at delivery• Placental insufficiency in utero• Maternal GDM• Dehydration• Idiopathic

• Rehydration

• Partial exchange transfusion

Thrombocytopenia

• Increased consumption• Immune (PLA-1 antibody)• Sepsis, DIC, TORCHS• Vasculopathic (hemangiomas)

• Rarely decreased production• TORCHS

• Rarely loss• Exchange transfusion

Metabolic Emergencies

• Hypoglycemia• Neonates tolerate lower glucose

concentration in the first few days• Nonspecific result of physiologic stress

• Prematurity, sepsis, asphyxia, polycythemia

• Specific result of metabolic disorders• Galactosemia, glycogen storage disease, AA

disorders, mitochondrial disease• Hyperinsulinemia

• GDM mother, Beckwith-Wiedemann Syndrome

Hypoglycemia

• Manifestation• Lethargy, jitteriness, seizure, apnea

• Management• Acute treatment

• 0.25-0.5 gm/kg, e.g. 2.5-5 ml/kg D10W• Glucagon 0.025 mg/kg IM (max 1 mg)

• Little role since lack of stores, especially if SGA

• Maintenance goal• 4-6 mg/kg/min (hence D10W, not D5W)

• Address underlying cause

Metabolic Emergencies

• Hypocalcemia• Early (<72 hours)

• Preemies• DiGeorge Syndrome• Infants of GDM mothers• Birth asphyxia

• Late (end of first week)• High PO4 containing formulas• Hypomagnesemia• Hypoparathyroidism

Hypocalcemia

• Manifestation• Lethargy, jitteriness, seizure, laryngospasm,

tetany; prolonged QTc

• Management• Acute treatment

• Ca gluconate (10%)• 1-3 ml/kg, 1ml/minute lest bradycardia

• Address underlying cause

Metabolic Emergencies

• Hyponatremia and hyperkalemia• Think congenital adrenal hyperplasia• Look for female virilization• Salt-wasting crisis can occur as neonate• DDx

• Gastroenteritis• Pyloric stenosis

• Hypochloremic metabolic alkalosis• +/- hyponatremia• +/- hypokalemia

CAH

• Management• ABCD’s• Work-up

• Serum cortisol, aldosterone, 17-OHP

• Glucocorticoid and mineralocorticoid replacement

• 2 mg/m2 Dexamethasone vs 100 mg/m2 Hydrocortisone

• Admit

Vomiting

• Causes• Infection

• Gastroenteritis, NEC, septicemia, meningitis, and urinary tract infections

• Milk allergy• Obstruction (if bile, think volvulus)

• Congenital anomalies (e.g. CDH, malrotation)

• Metabolic• Adrenal hyperplasia of the salt-losing variety, galactosemia,

hyperammonemias, organic acidemias

• Increased intracranial pressure

Constipation

• 90% pass meconium in the 1st 24hrs of life

• If not, or if constipation during neonatal period• Hirschsprung’s• CF• Hypothyroidism• Anal stenosis

Neonatal Seizures

• Atypical manifestation• Immature cortical organization and myelination

• Focal seizures with general insult• Electroclinical dissociation common• Common subtle presentations

• Lip smacking/chewing• Pedaling• Eye deviation• HR changes

Perinatal Causes

• HIE• Hemorrhage

• Intraventricular vs subarachnoid• Infection

• TORCHS included• Metabolic

• Hypoglycemia, hypocalcemia, hyponatremia• Pyridoxine deficiency

• Cerebral malformation• Trauma• Drug withdrawal

Management

• ABCD’s• FSWU

• CBCd, C+S (blood, urine, CSF), CXR

• Metabolic screen• Blood pH, Ca, PO4, sugar, electrolytes, renal

function, NH3

• CNS imaging• Address abnormalities• Benzodiazepines usually effective• Phenobarbital, phenytoin second line

Lessa G

• 2 week old girl born at term

• Lethargic

• No symptoms

• No signs until Neuro exam• Babinksi present

• Is this significant?

Lethargy

• Top of differential?• Infection• Neurologic injury or anomaly• Metabolic disorder

The Misfits

• T—Trauma/non accidental trauma• H—Heart disease (congenital)/Hypovolemia• E—Electrolyte disturbances• M—Metabolic disturbances• I—Inborn errors of metabolism• S—Sepsis• F—Formula dilution or over concentration• I—Intestinal catastrophes• T—Toxins (home remedies)• S—Seizures/CNS abnormalities

Lethargy

• Critically ill until proven otherwise• ABCD’s• FSWU

• CBCd, C+S (blood, urine, CSF), CXR

• Metabolic screen• Blood pH, Ca, PO4, sugar, electrolytes, renal

function, NH3

Last etiology to R/O – Sepsis

• Treatment of sepsis empiric• GBS, E.coli, Listeria• Staph, Strep

• Amp and Gent vs Amp and Cefotax

• TORCH• Treatment not always possible• Avoidance sometimes is

Irritability

• Think pain• Surgical problems• Trauma

• Similar DDx to lethargy• “The Misfits”

• A bit young for colic, needs an explanation

Respiratory distress

• Tachypnea• Increased work of breathing• Grunting

• Auto-PEEP, suggests primary pulmonary problem

• Can be due to systemic problem (e.g. infection)

• Tachycardia• Cyanosis

• Etiology• Pulmonary

• Hypoxia, aspiration, pneumothorax, CDH

• Cardiovascular• CHF (CHD, dysrhythmia), anemia

• Infection• Pneumonia, bronchiolitis, sepsis

• Metabolic• Hypoglycemia, hypocalcemia, hypothyroidism,

acidosis

Cyanosis

• Manifest when >5 gm/dL deoxyHgb• Normal Hgb 13-20

• Peripheral• Acrocyanosis, perioral• Common, can reflect vasomotor instability• Cool ambient temperature, shock, CHF

• Central• Mucous membrane, trunk, and extremity involvement• Etiology typically cardiac or pulmonary, occasionally

hypoventilation• Hyperoxia test

• PaO2 <100 in FiO2 100% suggests R-L shunt or mixing CHD

• Cyanotic heart diseases• Not all present with CHF

• Key diseases• Tetralogy Of Fallot• Transposition of the Great Arteries• Tricuspid atresia• Total Anomalous Pulmonay Venous Return• Truncus arteriosus• Hypoplastic Left Heart• Pulmonary atresia• Ebstein’s Anomaly

• Management of cyanotic CHD• ABCD’s• ECG, CXR, et al. labs

• Hyperoxic test

• PGE1 0.05-0.1 mcg/kg/min• Maintain or reopen PDA• Pulmonary and systemic vasodilator• Side effects of note – seizure, hypotension, apnea,

fever

Apnea

• Definition of Apnea• Respiratory pause

• >20 seconds OR…• Associated bradycardia, cyanosis, pallor, or

hypotonia

• Hypoxia, hypercarbia• Risk of cor pulmonale, hypertension, FTT• CNS effects not clear

Apnea

• Apnea of Prematurity• Obstructive (with inspiratory effort)• Central (without inspiratory effort)• Mixed

• Diagnosis of exclusion• < 30 weeks GA 80%• 30 - 31 weeks GA 50%• 32 - 33 weeks GA 14%• 34 - 35 weeks GA 7%

Apnea

• Apnea of prematurity• Management if several a day, or severe

• Trial of xanthines• Doxapram infusion• CPAP• Ventilation

ALTE

• Definition• “an episode that is frightening to the observer

and is characterized by some combination of apnea, color change, change in muscle tone, choking, or gagging.”

• NIH concensus group, Pediatrics 1987

• NOT “near-miss SIDS”

ALTE

• Incidence unknown• Heterogeneity of definitions, causes

• Most occur with infant awake• Etiology

• As many as 30-50% of ALTEs idiopathic• Aka “Apnea of Infancy”

Causes of ALTE, A’s and B’s• Digestive

• GER, esophagitis, aspiration, BM, perforation, malformation• Neurologic

• Seizure, ICH, HIE, malformation, hydrocephalus, hyperthermia, hypothermia, immaturity of respiratory center, sleep state

• Infection• Sepsis, meningitis, pneumonia, bronchiolitis, pertussis, NEC, UTI

• Respiratory• Airway anomaly, pneumothorax, laryngospasm, alveolar hypoventilation

• Cardiovascular• CHD, arrhythmia, anemia, CHF, shock, PFC, vasovagal

• Metabolic• Hypoglycemia, hypocalcemia, hyponatremia, hypernatremia, acidosis,

food intolerance, inborn errors• Miscellaneous

• Trauma (NAT), Munchausen by proxy, drugs

Alan B. Tse

• 4 week old ex34 week preemie

• Home one day, presents with apnea

• Approach• ABCD’s

• Stable

Approach to Al Tse

• Elements of history• Details of event

• True apnea? – not clear (it never is), maybe dusky• Intervention – stimulation, “but he’s on Caffeine”• Activity – awake, not distressed, no motor activity• Recently fed• Back to normal after event

• Perinatal Hx, ROS, Fam Hx• Infection risk, etc.

Handling Al Tse

• Focus of physical• Cardiovascular• Pulmonary• Neurological

Managing Al Tse

• Work-up as indicated• Might be similar to that for lethargy• Correct abnormalities as able

• Disposition• Monitored admission

• No serious events during hospitalization

• Parents’ burning question• “How do we watch for this at home?”

Home Monitoring

• Steinschneider in 1972 documented apnea in two siblings who later died of SIDS under the care of their mother

• Home monitoring advocated for 20 years thereafter

Home Monitoring

• Uncontrolled studies done (no RCTs), haven’t shown effectiveness• No epidemiologic evidence that monitors affect

incidence of SIDS• No evidence that ALTEs are precursors to SIDS• No evidence that monitors are used in cases at risk

for apnea or bradycardia (where they might be indicated)

• No evidence that monitors give enough warning for timely intervention, or that interventions would be effective

CHIME

• Collaborative Home Infant Monitoring Evaluation Study• 1,079 infants, 718,000 hours of monitoring

• Ramanathan et al, JAMA 2001

• Conventional apnea and bradycardia and extreme apnea and bradycardia are relatively common events, even among healthy term infants.

• Preterm infants had an increased risk of such events compared with healthy term infants, but only up to 43 weeks' postconceptional age

• The peak incidence of SIDS is more than 43 weeks' postconceptional age for preterm infants of any gestational age

• The evidence suggests that prolonged apnea and bradycardia are not immediate precursors of SIDS

SIDS

Crib death, cot death

• Definition• Sudden Unexpected Death (SUD) of a

previously healthy infant <12 months old• Unexplained after

• Autopsy (Medical Examiners case)• Within 24 hours• Skeletal survey, metabolic and toxicologic screen

• Examination of death scene• Review of medical records or clinical history

Theory

• Etiology unknown by definition

• Theories…• Multifactorial• Numerous “Triple Risk Hypotheses”

• Autonomic dysfunction – e.g. arcuate nucleus in brainstem underdeveloped

• Neurotransmitter anomalies• Astrogliosis

• Inconsistent evidence, i.e. cause vs effect• Guntheroth et al, Pediatrics 2002

A Theory of SIDS

The CPS Theory of SIDS• SIDS occurs during sleep • During sleep, the infant faces certain challenges ( airway

obstruction, decreased heart rate and blood pressure, a period when breathing stops or the rebreathing of CO2 when something pockets around the airway

• Research has shown subtle differences in the brainstem of SIDS infants which normally trigger the 'alarm system'

• a normal baby's alarm system comes into play when faced with challenges or stressors

• a SIDS baby's alarm system does not seem to detect CO2 increases, decreased O2 levels, decreased heart rate or airway closure

• Therefore, the goal of the risk reduction program is to interrupt the outside stressors in order to reduce the chance of SIDS. Medical research is now focussed on the vulnerable infant and the critical development period.

Differential

• Causes of SUD• SIDS• meningitis, sepsis, aspiration, pneumonia• myocarditis, significant congenital lesions,

arrhythmias (long QT)• dehydration, fluid and electrolyte imbalance, inborn

metabolic disorders• carbon monoxide asphyxia, drowning, burns• alcohol, drug, toxic exposure• abdominal or other trauma, NAT

Non-Accidental

• <5% of SIDS end up being discovered to have been abuse

• Increasing proportion as incidence of SIDS falls

• Autopsies cannot distinguish between asphyxiation (intentional or not) or SIDS

Non-Accidental

• Covert video recordings of life-threatening child abuse: lessons for child protection

• Southall DP, et al. Pediatrics. 1997

• Of 39 cases of investigated recurrent ALTEs, 33 were found to be abuse victims

• 30 had documented observations of intentional suffocation

• 12 of their 41 siblings had suffered SUD, 11 diagnosed as SIDS; 8 were later admitted to be from suffocation

Epidemiology of SIDS

• Peak 2-4 month olds

• 90% of SIDS <6 months old• 2% SUD >12 months old also unexplained

• In Canada• Third commonest cause of infant death

• Congenital anomalies, premature complications

• Commonest cause in 1-12 month olds• 3 per week in Canada – 1/2000 liveborns

• Relatively higher incidence in aboriginal population

Effects of Interventions

• Impact of 1993 statement and 1999 “Back-To-Sleep” campaigns in Canada• 385 diagnoses of SIDS 1989, 269 in 1994, and 138 in

1999• In Ontario, 40% of caregivers before and 71% after

campaign placed their babies supine to sleep

• All evidence of effectiveness is observational• Case-control• Following advice campaigns

• National and local

“Back To Sleep”

• Plenty of observational studies

• Benefits• Incidence of SIDS falls• Most significant modifiable risk factor• Supine > side > prone

“Back To Sleep”

• Risks• Healthy babies do not choke when supine

• Exceptions: Pierre-Robin and airway problems…• Malloy et al. Pediatrics 2000

• Plagiocephaly risked• “Tummy time” required for normal development• As develops motor skills, can find own comfortable

sleeping position• Kane et al. Pediatrics 1996

Tobacca Smoke avoidance

• Component of several observational studies

• Additional advice as part of campaigns• Not just an independent factor

• Lack of smoke not harmful• Second highest modifiable risk factor for SIDS

Tobacco Smoke avoidance

• Intrapartum smoke exposure is related to increased incidence of SIDS• Other intrapartum drugs not clear but

suspected

• Post-partum smoking definitely is too• Meta-analysis: Anderson et al. Health effects of passive

smoking. 2: Passive smoking and sudden infant death syndrome: review of the epidemiological evidence. Thorax 1997

• Other drug (e.g. EtOH) use not clear but suspected

Sleeping Surface

• No direct evidence• Sheepskin bedding a possible concern

• NZ Cot Death Study Group, J Pediatr1998

• Recommendation• Firm flat mattress best

• Related to over wrapping

Over heating or wrapping

• Advice as component of national campaigns• Not clearly independent factor

• No evidence of harm from NOT bundling

• Recommendation• Dress for comfort• Do not overheat, do not restrict• No pillows, stuffed toys, plastic wraps…

Bed-sharing

• One observational study felt this was an attributable risk• Not independent of maternal smoking• Attributable risks

• Maternal smoking alone 44% (OR 5.17)• Maternal smoking plus bed-sharing 33% (OR 11.1)

• Mitchell EA et al. Pediatrics 1997

• Another study found association between younger age of SIDS with bed-sharing, “particularly if the parent is large”

• Carroll-Pankhurst et al. Pediatrics 2001

Bed-sharing

• Most observational studies don’t show this to be an independent risk factor

• CPS concensus still• Bed-sharing does NOT impact incidence of

SIDS• Exceptions might include if drugs or EtOH

involved• N.B. Parents’ bed is a poor choice of surface

Breast-feeding

• Not good evidence• Part of the parcel of advised interventions

• Alm B et al. Arch Dis Child 2002

• But combined with other suspected benefits…

Soother Use

• Statistical reduction of SIDS with soother use

• Not strong enough association for promoting soother use• Abstract, Zotter et al. Wien Klin Wochenschr 2002

Risk Factors (CPS)• Babies who sleep on their tummies (6.6x) • Babies who sleep on their sides (2x) • Smoking during pregnancy (3x) • Exposure to second-hand smoke (2x) • Overheating • Cluttered sleeping area • Soft sleeping surface (increases with tummy sleeping) • Boys slightly more than girls • Aboriginal (3x) • Substance abuse during pregnancy • Teen mothers (less than 20 yrs of age) • Mothers with late or no prenatal care • Preterm infants (before 37 weeks gestation) • Low birthweight infants (under 2500 g) • Multiples (twins, triplets, etc.) • Mild respiratory infections • Unaccustomed tummy sleepers (18-20x)

Not proven risks

• Recurrent cyanosis

• Rates of apnea• 757 cases to 1514 BW matched controls

• National Institute of Child Health and Human Development data, 1988

• ALTE history• Reported in <10% of SIDS

Not proven risks

• Sibling• No conclusive data, most of it prior to 1990s• Twin’s RR=1.13 (95% CI, 0.97-1.31), second

twin dying of SIDS RR=8.17 (90% confidence interval, 1.18-56.67)

• 23,464 singleton SIDS deaths and 1,056 twin SIDS deaths

• Malloy MH et al. Pediatrics 1999

More Unproven risks

• Viral syndromes, URTIs• Maternal EtOH use• Caffeine use for A’s and B’s• Vaccination status

• ‘…the author of the study, concluded that “vaccination is the single most prevalent and most preventable cause of infant deaths.”’ (n=1; 1991)

• ‘A study published in JAMA found that children diagnosed with asthma (a respiratory ailment not unlike SIDS) were five times more likely than not to have received pertussis vaccine.’

Clinical management

• EMS trained to make observations• Position of the infant, type of bed or crib and any

defects, amount and position of clothing and bedding • Marks on the body, body temperature and rigor, room

temperature, type of ventilation and heating• Terminal motor activity (e.g. clenched fists,

postmortem anal dilation), mottling or postmortem lividity, oronasal d/c

• Reaction of the caregivers

In the ED

• ABCD/DOA as indicated• Diagnosis = “Probable SIDS”

• Explain to the family the need for thorough investigation to explain this SUD

• Involvement of• Social worker +/- pastoral care• Law enforcement officer• Medical examiner

• Pathologist• Child abuse expert

• Work-up as described by: AAP Committee on Child Abuse and Neglect, Pediatrics 2001

In the ED

• History• Non-accusatory, empathic – it will be stressful• HPI

• Setting, recent feed, position when put to sleep vs when found, CPR performed, caregivers and other children around, EMS findings

• ROS• General health, recent infection, cardiopulmonary status,

GER, seizures, ALTEs• PMHx

• Perinatal course, growth and development• FamHx

• Lost pregnancies, SUD, congenital problems, consanguinity, medication or drug exposure

In the ED

• History• Suspicious findings

• Age at death older than 6 months• Previous recurrent cyanosis, apnea, or ALTE while in the

care of the same person• Discovery of blood on the infant's nose or mouth in

association with ALTEs• Previous unexpected or unexplained deaths of 1 or more

siblings• Simultaneous or nearly simultaneous death of twins• Previous death of infants under the care of the same

unrelated person

Lesson Learnt

• The mother Steinschneider was involved with had had 3 other infants die previously under her care

• She was successfully convicted of murder x5 more than 20 years after the fact