ALTE, SIDS, and Diseases of Prematurity

ALTE, SIDS, and Diseases ALTE, SIDS, and Diseases of Prematurityof Prematurity

ALTE, SIDS, and Diseases ALTE, SIDS, and Diseases of Prematurityof Prematurity

Chris McCrossin, PGY 3

Thanks to: Kelly Millar, Bella Sztukowski, Ian Wishart, and Jay Green

Chris McCrossin, PGY 3

Thanks to: Kelly Millar, Bella Sztukowski, Ian Wishart, and Jay Green

objectivesobjectives

Understand the underlying etiologies for ALTE’s, what to look for on history and physical exam

Gain an appreciation for what we understand as the natural history and future risk to those presenting with ALTE’s and how it compares and contrasts to SIDS

Know what constitutes an appropriate ED evaluation, work-up, and disposition of ALTE cases

SIDS (definitions, addressing parental questions, appropriate recommendations, identify risk in future siblings)

Diagnoses associated with prematurity:

Bronchopulmonary dysplasia

SAH

NEC

Understand the underlying etiologies for ALTE’s, what to look for on history and physical exam

Gain an appreciation for what we understand as the natural history and future risk to those presenting with ALTE’s and how it compares and contrasts to SIDS

Know what constitutes an appropriate ED evaluation, work-up, and disposition of ALTE cases

SIDS (definitions, addressing parental questions, appropriate recommendations, identify risk in future siblings)

Diagnoses associated with prematurity:

Bronchopulmonary dysplasia

SAH

NEC

CasesCases

1. 3 week old infant, chokes on a vitamin D tablet, turns purple. Parents saw her go limp and describe a period of apnea, mom gives 5-6 assisted respirations, baby looks well on arrival in ED

2. 6 week old male, asleep in his car seat, dad glanced down at him and noticed him to be bluish and not breathing. Parents give assisted respirations while waiting for EMS. Baby still listless on EMS arrival but vitals stable in ED

3. 3 month old female, referred in to ED because twin sister dies that AM from SIDS. She’s entirely asymptomatic




DefinitionsDefinitions

ALTE (Apparent Life Threatening Event)

An episode that is frightening to the observer and is characterized by a combination of:

Apnea

Color change

• Marked change in muscle tone

• Choking

• Gagging

ALTE (Apparent Life Threatening Event)

An episode that is frightening to the observer and is characterized by a combination of:

Apnea

Color change

• Marked change in muscle tone

• Choking

• Gagging

DefinitionsDefinitions

SIDS

Sudden death of an infant < 1 year old

Remains unexplained after investigations including

Complete autopsy

Examination of the death scene

A review of the clinical history

SIDS

Sudden death of an infant < 1 year old

Remains unexplained after investigations including

Complete autopsy

Examination of the death scene

A review of the clinical history

SIDS EpidemiologySIDS Epidemiology

95% < 6-8 mo

Peaks 2-4 months

1% < 1 mo, 2% > 2 years

How effective is the “Back to sleep campaign?”

Before: ~ 7000 deaths/year in the U.S. (1.3/1000)

After: ~ 2300 deaths/year in the U.S. (0.57/1000)

95% < 6-8 mo

Peaks 2-4 months

1% < 1 mo, 2% > 2 years

How effective is the “Back to sleep campaign?”

Before: ~ 7000 deaths/year in the U.S. (1.3/1000)

After: ~ 2300 deaths/year in the U.S. (0.57/1000)

Nelson’sNelson’s

SIDS Risk FactorsSIDS Risk Factors

Modifiable

Cigarette smoking

Sleep Environment

Drug and Ethanol Use

Pregnancy Related

Nutritional

Non-Modifiable

Socioeconomic status

• Prematurity

Genetic

Modifiable

Cigarette smoking

Sleep Environment

Drug and Ethanol Use

Pregnancy Related

Nutritional

Non-Modifiable

Socioeconomic status

• Prematurity

Genetic Nelson’sNelson’s

Recurrent SIDS in a SiblingRecurrent SIDS in a Sibling

Idiopathic

CNS Congenital central hypoventilation, Neuromuscular disorders

Cardiac Endocardial fibroelastosis, WPW, prolonged QT, Congenital heart block P

Pulmonary Pulmonary Hypertension

Endocrine/Metabolic

Extensive list - watch for consanguinity

Infection Disorders of immune host defense

Child Abuse Infanticide, Munchausen by proxy

Genetic Risk of death in a twin ~ 2 x the risk

Nelson’sNelson’s

ALTE EpidemiologyALTE Epidemiology

What % of patients who die from SIDS have had a previous ALTE?

7-8%

% of ALTE’s who subsequently die unexpectedly?

10% in ALTE’s occuring during sleep + require CPR

What time do ALTE’s tend to occur?

During Waking hours

At what age do children with ALTE’s present?

2 months on average

Risk Factors for ALTE?

Maternal smoking, single parents, nocturnal diaphoresis

What % are eventually considered idiopathic?

50%

What is the recurrence rate for severe ALTE?

Up to 68% in some studies!

What % of patients who die from SIDS have had a previous ALTE?

7-8%

% of ALTE’s who subsequently die unexpectedly?

10% in ALTE’s occuring during sleep + require CPR

What time do ALTE’s tend to occur?

During Waking hours

At what age do children with ALTE’s present?

2 months on average

Risk Factors for ALTE?

Maternal smoking, single parents, nocturnal diaphoresis

What % are eventually considered idiopathic?

50%

What is the recurrence rate for severe ALTE?

Up to 68% in some studies!

ALTE OutcomesALTE Outcomes

Retrospective cohort

N = 471

Urban setting

Excluded patients with probable etiologies at time of first presentation

5 year ave follow-up (range: 2-7 years)

Retrospective cohort

N = 471

Urban setting

Excluded patients with probable etiologies at time of first presentation

5 year ave follow-up (range: 2-7 years)

Pediatrics 2008; 122(1): 125-31Pediatrics 2008; 122(1): 125-31

ALTE vs SIDSALTE vs SIDS

Common Risk Factor: Smoking during pregnancy

Differences: Most ALTE’s occur in peds < 2 months, compared with a 2-4 month peak for SIDS

52% of ALTE’s occur during wakefulness

SIDS occurs during sleep

No change in ALTE incidence with back to sleep program

Common Risk Factor: Smoking during pregnancy

Differences: Most ALTE’s occur in peds < 2 months, compared with a 2-4 month peak for SIDS

52% of ALTE’s occur during wakefulness

SIDS occurs during sleep

No change in ALTE incidence with back to sleep program

J Pediatr 2009; 154(3): 317-19J Pediatr 2009; 154(3): 317-19

WhIch ALTE’s are At Higher Risk?WhIch ALTE’s are At Higher Risk?

Infants born prematurely

Infants < 43 weeks post-conceptional age

ALTE’s that occur with symptoms of URTI’s

Infants born prematurely

Infants < 43 weeks post-conceptional age

ALTE’s that occur with symptoms of URTI’s

J Pediatr 2009; 154(3): 332-37J Pediatr 2009; 154(3): 332-37

Critical DiagnosisCritical Diagnosis

Bronchiolitis (12%)

Seizures (8%)

Sepsis (7%)

ICH (3%)

Meningitis (2%)

Dehydration (2%)

Anemia (2%)

Bronchiolitis (12%)

Seizures (8%)

Sepsis (7%)

ICH (3%)

Meningitis (2%)

Dehydration (2%)

Anemia (2%) Ann Emerg Med 2004: 43:711-17Ann Emerg Med 2004: 43:711-17

Differential DiagnosisDifferential Diagnosis

ApneaApnea

Most common sign associated with ALTE

Apnea can be normal or pathologic

Various Terms:

Apnea of infancy - unexplained pathologic apneic event occurs for the first time in an infant older than 37 weeks (e.g. unexplained ALTE)

Apnea of prematurity - occurs prior to 37 weeks at which point it resolves

Periodic breathing - three or more resp pauses of greater than 3 seconds duration with less than 20 seconds of respiration between pauses. Common and physiologic in preterm infants. considered pathologic if associated with cardiorespiratory instability

Most common sign associated with ALTE

Apnea can be normal or pathologic

Various Terms:

Apnea of infancy - unexplained pathologic apneic event occurs for the first time in an infant older than 37 weeks (e.g. unexplained ALTE)

Apnea of prematurity - occurs prior to 37 weeks at which point it resolves

Periodic breathing - three or more resp pauses of greater than 3 seconds duration with less than 20 seconds of respiration between pauses. Common and physiologic in preterm infants. considered pathologic if associated with cardiorespiratory instability

Pathologic ApneaPathologic Apnea

no air movement for > 20 seconds* OR

any period of no air movement associated with physiologic compromise (bradycardia, pallor, hypotonia, cyanosis)

Central

No drive from resp centers, neuromuscular insufficiency

e.g. head trauma, Ondine’s curse, apnea of prematurity

Obstructive

Breathing through an occluded airway

e.g. masses, adenotonsillar hypertrophy, OSA, foreign body, laryngomalacia, intralumial cysts

Mixed

Two conditions; e.g. premature infant with central apnea and nasal congestion from URTI,

One condition with features of both; e.g. GERD

no air movement for > 20 seconds* OR

any period of no air movement associated with physiologic compromise (bradycardia, pallor, hypotonia, cyanosis)

Central

No drive from resp centers, neuromuscular insufficiency

e.g. head trauma, Ondine’s curse, apnea of prematurity

Obstructive

Breathing through an occluded airway

e.g. masses, adenotonsillar hypertrophy, OSA, foreign body, laryngomalacia, intralumial cysts

Mixed

Two conditions; e.g. premature infant with central apnea and nasal congestion from URTI,

One condition with features of both; e.g. GERD

InfectionsInfections

One retrospective analysis suggested that SBI’s occurred in close to 3% of well appearing infants presenting with ALTE (risk is higher for premature infants)

Newborns may show little in terms of signs/symptoms of serious bacterial infections so go looking for it!

One retrospective analysis suggested that SBI’s occurred in close to 3% of well appearing infants presenting with ALTE (risk is higher for premature infants)

Newborns may show little in terms of signs/symptoms of serious bacterial infections so go looking for it!

Ped Emerg Care 2009; 25(1):19-25Ped Emerg Care 2009; 25(1):19-25

GERDGERD

One of the most common diagnoses made in the setting of ALTE

Unclear if GERD is purely and associative, responsive, or triggering factor in ALTE’s

One of the most common diagnoses made in the setting of ALTE

Unclear if GERD is purely and associative, responsive, or triggering factor in ALTE’s

J Pediatr 2000; 137(3): 298-300J Pediatr 2000; 137(3): 298-300Ped Clin N Am 2005; 52(4): 1127-46/ Ped Clin N Am 2005; 52(4): 1127-46/

HistoryHistory

Identify central apnea vs obstructive apnea if possible (respiratory pause vs choking, gagging, or gasping)

Try to recreate a technicolor image in your mind what the caregiver saw, what the infant was doing before the event, what happened immediately after, and the infant’s position at the time

Read the EMS report: What did the infant look like when they arrived?

Identify central apnea vs obstructive apnea if possible (respiratory pause vs choking, gagging, or gasping)

Try to recreate a technicolor image in your mind what the caregiver saw, what the infant was doing before the event, what happened immediately after, and the infant’s position at the time

Read the EMS report: What did the infant look like when they arrived?

HistoryHistory

A good feeding history will identify cardiac causes, TEF, and swallowing dysfunction

Ask about snoring, look for obstructions, query about foreign body aspiration

Family history: ask about siblings

A good feeding history will identify cardiac causes, TEF, and swallowing dysfunction

Ask about snoring, look for obstructions, query about foreign body aspiration

Family history: ask about siblings

Physical ExamPhysical Exam

Good CVS, Resp, Abdo, and Neuro Exams

Look for signs of trauma

Don’t miss the fundoscopic exam

Good CVS, Resp, Abdo, and Neuro Exams

Look for signs of trauma

Don’t miss the fundoscopic exam

Investigations Investigations

Basic screening tests

CBC, CH6, ABG, Ammonia level, CXR, ECG

Consider septic work-up

Consider Tox screen

Targeted testing based on history and symptoms

Basic screening tests

CBC, CH6, ABG, Ammonia level, CXR, ECG


Consider Tox screen

Targeted testing based on history and symptoms

CasesCases







DispositionDisposition

Choking episodes

Clear choking episodes are not usually life-threatening

Assuming no hx of chronic feeding problems monitor for a few hours, ensure a normal feed occurs, then discharge home

ALTE

If true apnea or significant resuscitation in field most will admit for monitoring (PICU)


Choking episodes

Clear choking episodes are not usually life-threatening

Assuming no hx of chronic feeding problems monitor for a few hours, ensure a normal feed occurs, then discharge home

ALTE

If true apnea or significant resuscitation in field most will admit for monitoring (PICU)


Sids in the EDSids in the ED

Twins of SIDS deaths are admitted for monitoring

Rare that a SIDS death will be brought to ED in active resuscitation

Labs in this case may be helpful for prevention of future siblings

Twins of SIDS deaths are admitted for monitoring

Rare that a SIDS death will be brought to ED in active resuscitation

Labs in this case may be helpful for prevention of future siblings

Minimizing SIDSMinimizing SIDS

Canadian Pediatric Society Recommendations:

Babies should sleep on their back for the first year of life (or until they can turn over on their own)

Firm surface

Soft material out of baby’s sleep environment

Make sure baby is not too warm

Keep baby away from cigarette smoke

No bed sharing

Risk of SIDS with bed sharing is increased if parent has had alcohol, taken any drug with sedating side effects

Canadian Pediatric Society Recommendations:

Babies should sleep on their back for the first year of life (or until they can turn over on their own)

Firm surface

Soft material out of baby’s sleep environment

Make sure baby is not too warm

Keep baby away from cigarette smoke

No bed sharing

Risk of SIDS with bed sharing is increased if parent has had alcohol, taken any drug with sedating side effects

Take-Home PointsTake-Home Points

SIDS and ALTE’s are not the same

ALTE’s are primarily a result of Apnea

Most children with ALTE’s do fine but severe ALTE’s are at higher risk

Although ALTE’s are idiopathic 50% of the time, remember your differential

Think about Sepsis

Think about child abuse

The most important modifiable risk factor for SIDS that we know about is ensuring a safe sleep environment (back to sleep)

SIDS and ALTE’s are not the same

ALTE’s are primarily a result of Apnea

Most children with ALTE’s do fine but severe ALTE’s are at higher risk

Although ALTE’s are idiopathic 50% of the time, remember your differential

Think about Sepsis

Think about child abuse

The most important modifiable risk factor for SIDS that we know about is ensuring a safe sleep environment (back to sleep)

You thought you were having a bad day...You thought you were having a bad day...

...and then it got worse...and then it got worse

PrematurityPrematurityPrematurityPrematurity

PrematurityPrematurity

Definitions

Late preterm - GA greater than 34, less than 37 weeks

Very preterm - GA less than 32 weeks

Low birth weight - Less than 2500g

Very low birth weight - Less than 1500g

Extremely low birth weight - Less than 1000g

Complications with prematurity

RDS 44%

• PDA 30%

BPD 20%

Late onset sepsis 20%

IVH 12%

NEC 7%

Periventricular leukomalacia 5%

Definitions

Late preterm - GA greater than 34, less than 37 weeks

Very preterm - GA less than 32 weeks

Low birth weight - Less than 2500g

Very low birth weight - Less than 1500g

Extremely low birth weight - Less than 1000g

Complications with prematurity

RDS 44%

• PDA 30%

BPD 20%

Late onset sepsis 20%

IVH 12%

NEC 7%

Periventricular leukomalacia 5% www.uptodate.comwww.uptodate.com

CASECASE

16 day old infant presents to the ED with 2-3 days of vomiting. Had been doing well with feedings prior to that. Parents now feel that the vomiting is becoming increasingly forceful

Vomiting becoming dark brown/maroon in color

Last few stools have become darker than usual

No BM, No wet diapers in past 8 hours

No fevers, no sick contacts

Went to WIC yesterday, dx with “overfeeding”

PMHx

born @ 34 weeks gestational age

1 week in NICU requiring phototherapy for hyperbilirubinemia

No pulmonary or cardiovascular issues

16 day old infant presents to the ED with 2-3 days of vomiting. Had been doing well with feedings prior to that. Parents now feel that the vomiting is becoming increasingly forceful

Vomiting becoming dark brown/maroon in color

Last few stools have become darker than usual

No BM, No wet diapers in past 8 hours

No fevers, no sick contacts

Went to WIC yesterday, dx with “overfeeding”

PMHx

born @ 34 weeks gestational age

1 week in NICU requiring phototherapy for hyperbilirubinemia

No pulmonary or cardiovascular issues

Necrotizing Necrotizing enterocolitisenterocolitisNecrotizing Necrotizing enterocolitisenterocolitis

NECNEC

Etiology

Unknown, multifactorial (ischemic/infectious insults/feeding related)

Spectrum of presentation

I - Early or suspected NEC based on feeding intolerance, vomiting, or ileus

II - NEC proven on AXR with abdominal dilation and pneumatosis intestinalis

III - Advanced disease with perforation, metabolic acidosis, DIC, shock

Etiology

Unknown, multifactorial (ischemic/infectious insults/feeding related)

Spectrum of presentation

I - Early or suspected NEC based on feeding intolerance, vomiting, or ileus

II - NEC proven on AXR with abdominal dilation and pneumatosis intestinalis

III - Advanced disease with perforation, metabolic acidosis, DIC, shock

Who’s at Risk?Who’s at Risk?

Prematurity

Low birth-weight

Timing of presentations:

Term infants less than 1 week old

Within first 3 weeks of life in infants born at 29-32 weeks GA

Between 2-4 weeks of life in infants born at 24-28 weeks GA

Prematurity

Low birth-weight

Timing of presentations:

Term infants less than 1 week old

Within first 3 weeks of life in infants born at 29-32 weeks GA

Between 2-4 weeks of life in infants born at 24-28 weeks GA

Clinical PresentationClinical Presentation

May appear well if early or may present in a profound state of shock

Non-specific signs/symptoms

feeding intolerance

blood in vomit or stool

apnea

respiratory distress

abdominal distention

May appear well if early or may present in a profound state of shock

Non-specific signs/symptoms

feeding intolerance

blood in vomit or stool

apnea

respiratory distress

abdominal distention

InvestigationsInvestigations

Labs are non specific but serve as markers of severe disease and follow trends

Thrombocytopenia

Neutropenia

Coagulopathy

CRP

Lactate

Blood Gases

Labs are non specific but serve as markers of severe disease and follow trends

Thrombocytopenia

Neutropenia

Coagulopathy

CRP

Lactate

Blood Gases

X-Ray FindingsX-Ray Findings

Dilated loops of bowel

Air fluid levels

Free air

Pneumatosis intestinalis

Portal venous gas

Dilated loops of bowel

Air fluid levels

Free air

Pneumatosis intestinalis

Portal venous gas

X-Ray FindingsX-Ray Findings

ManagementManagement

Who Needs Surgery?Who Needs Surgery?

Only hard indication is bowel perforation

“Soft” indications:

Radiological signs

persistent fixed loop

portal venous gas

ascites

Lab features

severe acidosis

Only hard indication is bowel perforation

“Soft” indications:

Radiological signs

persistent fixed loop

portal venous gas

ascites

Lab features

severe acidosis

Bronchopulmoary Bronchopulmoary DysplasiaDysplasia

Bronchopulmoary Bronchopulmoary DysplasiaDysplasia

DefinitionDefinition

Often used as a “catchall” term to describe chronic lung disease in the neonatal population

Clinical definition: requiring O2 @ 36 weeks postmenstrual age

Defining who needs supplemental O2 therapy is not black and white and practice varies widely

Often used as a “catchall” term to describe chronic lung disease in the neonatal population

Clinical definition: requiring O2 @ 36 weeks postmenstrual age

Defining who needs supplemental O2 therapy is not black and white and practice varies widely

J Perinat 2008; 28(12): 837-840J Perinat 2008; 28(12): 837-840

PathogenesisPathogenesis

May have severe or mild respiratory diseaseMay have severe or mild respiratory disease

Initially vented with low pressures and FiO2Initially vented with low pressures and FiO2

Honeymoon PeriodHoneymoon Period

After weeks may show progressive deterioration in lung function After weeks may show progressive deterioration in lung function

BPDBPDSem Neonat 2003; 8(1):

63-71

Sem Neonat 2003; 8(1):

63-71

Who is at Risk?Who is at Risk?

Most infants with BPD are born prematurely

75% weigh less than 1 kg at birth

20% of ventilated newborns

Most infants with BPD are born prematurely

75% weigh less than 1 kg at birth

20% of ventilated newborns

Lancet 2006; 367(9520): 1421-31Lancet 2006; 367(9520): 1421-31

Natural HistoryNatural History

Most infants with BPD will show progressive improvement in pulmonary function and wean from supplemental O2 as their lungs grow and remodel

50% of all infants with BPD will need to be readmitted to hospital during early childhood for respiratory distress often exacerbated by RSV

High rate of admission falls during the second year of life

Strong association between BPD and growth retardation and cognitive delays

Most infants with BPD will show progressive improvement in pulmonary function and wean from supplemental O2 as their lungs grow and remodel

50% of all infants with BPD will need to be readmitted to hospital during early childhood for respiratory distress often exacerbated by RSV

High rate of admission falls during the second year of life

Strong association between BPD and growth retardation and cognitive delays

Lancet 2006; 367(9520): 1421-31Lancet 2006; 367(9520): 1421-31

Radiographic FindingsRadiographic Findings

Hyperinflation

Non-homogeneity of pulmonary tissues

Densities extending to the periphery

Diffuse haziness

Hyperinflation

Non-homogeneity of pulmonary tissues

Densities extending to the periphery

Diffuse haziness

Sem Neonat 2003; 8(1): 63-71Sem Neonat 2003; 8(1): 63-71

ManagementManagement

Treat as per asthmatic pathway

Ventolin

Atrovent

Dexamethasone

Treat as per asthmatic pathway

Ventolin

Atrovent

Dexamethasone

www.uptodate.comwww.uptodate.com

Intraventricular Intraventricular HemorrhageHemorrhage

Intraventricular Intraventricular HemorrhageHemorrhage

intraventricular hemorrhageintraventricular hemorrhage

Bleeding originates from the germinal matrix

Occurs most frequently in infants born before 32 weeks or less than 1500g

Virtually all IVH occurs in the first 5 postnatal days

Bleeding originates from the germinal matrix

Occurs most frequently in infants born before 32 weeks or less than 1500g

Virtually all IVH occurs in the first 5 postnatal days

Risk FactorsRisk Factors

Prematurity

Vaginal delivery

Intrapartum asphyxia

RDS

Prolonged neonatal resuscitation

Acidosis

Prematurity

Vaginal delivery

Intrapartum asphyxia

RDS

Prolonged neonatal resuscitation

Acidosis

Clinical PresentationClinical Presentation

Two Types of Presentations:Two Types of Presentations:

DiagnosisDiagnosis

Ultrasound

Routine U/S screening in all infants with GA < 30 weeks

Screening @ 7 & 14 days of age and repeated @ 36-40 weeks post menstrual ages

LP

If no U/S available

Findings similar to SAH

Ultrasound

Routine U/S screening in all infants with GA < 30 weeks

Screening @ 7 & 14 days of age and repeated @ 36-40 weeks post menstrual ages

LP

If no U/S available

Findings similar to SAH

Post-Hemorrhagic HydrocephalusPost-Hemorrhagic Hydrocephalus

35% of infants with IVH

Management

Varies depending on severity

Serial U/S monitoring

Head circumference measurement

Surgery +/- shunt insertion

35% of infants with IVH

Management

Varies depending on severity

Serial U/S monitoring

Head circumference measurement

Surgery +/- shunt insertion

SummarySummary

Documents

ALTE, SIDS, and Diseases of Prematurity