altes, sids, and prems russell lam september 1, 2011 special thanks to bela sztukowski for her help...

ALTEs, SIDS, and Prems

Russell Lam

September 1, 2011

Special thanks to Bela Sztukowski for her help on this presentation

Objectives

Discuss the history of ALTEs, diagnostic work-up, and follow-up

Review risk factors for SIDS

Review some conditions commonly encountered in the ED relevant to prematurely born patients

Case 12 mo male brought in after a

choking episode Grandmother picked up baby

after a nap, 2 hours post feed Baby made choking noise

and turned off-colour. Back blows given

Vitals in ED: P120 R45 T 37 BP 95/60 Sp02 100% room air

Exam unremarkable

What investigations do you want (if any)?

How long will you monitor in the ED?

What do you tell this grandmother?

A historical perspective

“The hypothesis implicating prolonged apnea during sleep is causally related to SIDS underscores the need for further research directed toward a greater understanding of the variables influencing the occurrence of sleep apnea…”

2 decades later – evidence of infanticide for all 5 infants became known

Definitions

Apparent Life Threatening EventFrightening to the observerCombination of

Apnea Color change Tone change Cough or gagging

Infantile Apnea and Home Monitoring. NIH Consensus Statement 1986 Sep 29-Oct 1;6(6):1-10.

Definitions

Sudden infant death syndromeDeath of infant or child unexplained by historyPost mortem fails to demonstrate adequate

explanation

Less than 1 yearCase investigation and death scene examination fail

to demonstrate adequate explanation


Willinger M, James LS, Catz C. Defining the sudden infant death syndrome (SIDS): deliberations of an expert panel convened by the National Institute of Child Health and Human Development. Pediatr Pathol. 1991;11:677–684

Definitions

Apnea of infancyUnexplained cessation in breathing > 20s or < 20s if

Bradycardia Cyanosis Pallor Hypotonia

Apnea of prematuritySame as above but < 37 weeks GA


Who gets ALTEs?

0.5-6% of all infants

Difficult to estimate true incidence as:Subjective nature of definitionNot all ALTEs will visit the EDRetrospective data

Brooks JG. Apparent life-threatening events and apnea of infancy. Clin Perinatol 1992;4:809 – 838.

Who gets ALTEs?

Prospective study (1993-2001)2.46/1000 live birthsAverage age of ALTE = 8 weeks55% of ALTEs had diagnoses

Respiratory (RSV/pneumonia) (29%) GI (GERD/Feeding aspiration) (22%) Congenital cardiac (2%) Metabolic/Neuro (2%)

ALTE Risk Factors

Family history of infant death, single parenthood, profuse night sweating, smoking, repeated cyanotic episodes, pallor, apnea, feeding difficulties

Typical History

Breathing Apnea 70%

Difficulty breathing 62%

Colour Cyanosis 71%

Red face 29%

Pallor 51%

Tone Stiffness 46%

Floppiness 43%

Limb Jerking 22%

GI Choking 35%

Vomiting 18%

Typical physical

Stratton SJ, Taves A, Lewis RJ, et al. Ann Emerg Med 2004; 43:711–717

General physical appearance, work of breathing, circulatory signs, respiratory rate, pulse rate not clinically abnormal

Differential of the cause of an ALTE?

Causes of ALTEs?

N = 643 pts (1991-2002)

Most common diagnosesGERD (31%)Seizure (11%)LRTI (8%)Unknown (23%)

Serious Bacterial Infection?

Altman (2008) – Retrospective chart review N=243 5% had occult bacterial infection 26% had obvious bacterial infection

Mittal (2009) – Prospective cohort N=198 22.2% had cultures 0% had serious bacterial infection

Zuckerbraun (2009) – Retrospective chart review N=182 61.5% had cultures 2.7% had serious bacterial infection Premature patients more likely to have SBI (6.7 v 0.8%)

A reasonable work-up?

Back to the case…

Would you admit this 2 month old patient?History = consistent with ALTE definitionPhysical = normalRisk Factors

None (no smoke at home, usually feeds well, married parents)

3 year prospective study N = 59

8 patients met “hospital required” outcome criteriaMultiple ALTEs and prematurity (<37 weeks) SD from

“hospital not required patients”Most common demographic features were age <

1mos and multiple ALTEs

From this study, 2 criterion features developed:

age < 1mos and/or multiple ALTEs yields 100% NPV 100% Sens for need for hospital admission

Mortality? Recurrence?

9 year prospective study N = 563

3 deaths (0.5%)2 SIDS and 1 from child abuse

Recurrence37.9% had recurrent episodes8.9% would return visit for ALTE

How is ALTE different from SIDS?

Take home points on ALTE

Scary+ Apnea/Colour Change/Tone/Choking

Broad differential but mostly GER/LRTI/CNS

Likely need admission + broad work-up

Low mortality rate (0.5%)

ALTE ≠ SIDS

Case 2

6 mo male brought in because of cough x 3 days

You diagnose URTI and discharge the patient

On the way out, mother asks: “By the way, a mother from book club just got an apnea monitor. Should I get one too?”

A little about SIDS

Most common cause of death in 1mos-1y (20-25% of all deaths < 1 year)

2006 = 0.54 per 1000 live births in the US

Most will occurs age 2-4 months, almost all by 6 months

SIDS versus SUDI

Sudden Unexpected Death of Infancy (SUDI)Umbrella term which includes SIDS but also other

causes of sudden infant death (CVS, Abuse, Metabolics)

SIDS requires autopsy and death scene examination

Pathophysiology of SIDS

Filiano and Kinney. Biol Neonate 1994;65(3-4): 194-7

Long QT?

Schwartz et al. 1998. New Eng J Med. 338 (24):1709-1714

Risk factors?

Risk factors?

Sleeping prone

Maternal smoking during and after pregnancy

Bed-sharing, especially if EtOH or very tired parent

Soft bedding, pillow, covers over the head

Prematurity (<37 weeks)

Low birth weight (<2500g)

A safe sleeping environment

CPS 2004 Guidelines1st six months babies should sleep in own crib in

parent’s roomSleep on back in an approved cribNo quilts/comforters, pillowsRoom-sharing is protective, bed-sharing is notNo sleeping on couch, water bed, air mattress, car

seats

Pacifiers?

Huack et al. Pediatrics (2005). 116 (5):716-722

CPS (2004)Does not recommend pacifier use to reduce risk of

SIDSCaution before routinely advising against pacifier use

AAP (2005)Pacifier for 1st year of life when putting down to sleepDelay until 1 month of age if exclusive breastfeeding

Apnea Monitors?

CHIME study (1994-1998)1079 infants in 4 groups

Healthy Term, Idiopathic ALTE, SIDS-Sibling, Preterm All given plethysmography

All groups had similar numbers of apnea/bradycardia on monitors Extreme apneas in 10% of all infants Significantly more AsBs in Preterm infants

But all resolved by 43 weeks post conceptual age 6 deaths, none on monitors

Apnea Monitors?

AAP 2005Many infants get Apneas/Bradycardia and do not dieApnea resolves prior to when most SIDS deaths

occurDoes not prevent SUDIPossible groups who need apnea monitors

Preterm infants CPAP/Trach’d patients

Twins?

Malloy (1995)N = 23464 single SIDS deaths and 1056 twin SIDS

deathsRR 1.13 (95%CI 0.97-1.31) for twins when adjusted

for birth weightRR 8.17 (90%CI 1.18-56.67) if 1 twin died of SIDS

Getahun (2004)N = 501 SIDS deaths overallRR 1.9 (95%CI 1.68-2.01) but not matched for birth

weightRR 4.7 (95%CI not reported) if 1 twin died of SIDS

Take home points on SIDS

Different from ALTE

Two most important risk factors are prone sleeping and maternal smoking

Back to sleep in their own crib

Don’t discourage pacifiers

Apnea monitors don’t help

If a twin already died of SIDS, other twin at way higher risk. Admission debatable…

Case 3

2 month old ex 24 week male comes in with wheeze and cough

Mom hands you a summary from the NICU that she was givenELBW and SGARDS/BPDNECGrade III IVHROP Zone 2 Stage 1GERD with FundoG-Tube Fed

The Lingo

Prematurity = <37 weeks gestational age

Birth weightLow birth weight = < 2500gVery low birth weight = < 1500gExtremely low birth weight = < 1000g

The Lingo

Age Terminology During Perinatal Period. Pediatrics. 114 (5):1362-1364

Bronchopulmonary Dysplasia

Defined by oxygen needs beyond 28 days of life

Initial respiratory disease (RDS/Meconium Aspiration) then chronic lung disease that develops afterwards

3 big risk factorsOxygen toxicityMechanical ventilationExaggerated inflammatory response

Lacy Gomella. Neonatology. 2004

BPD pearls for the ED

Examines like asthma Increased RR, wheeze, crackles

Treat like asthmaSABA, Inhaled Corticosteroids, Oxygen

Consider diuretics

Special consideration: RSV If RSV and BPD, more likely to develop apnea and a

more severe course = admit!Passive RSV immunoglobulin upon NICU discharge?

Fleisher et al. Textbook of Pediatric Emergency Medicine. 2010

Necrotizing Enterocolitis

Spectrum of acquired neonatal disease with end expression of serious intestinal injury

Etiology is multifactorial Infectious/Ischemic/Feeds

Mostly in preterms in first few weeks of life but can present in term babies in first 10 days of life

Presents as Septic infant Lower GI Bleeding Abdominal distension and feed intolerence/vomiting

Hackam. Necrotizing Enterocolitis: A leading cause of death and disability. 2008. http://knol.google.com/k/dr-david-hackam/necrotizing-enterocolitis/Mpv6w2_G/t5RhXw.

Hackam. Necrotizing Enterocolitis: A leading cause of death and disability. 2008. http://knol.google.com/k/dr-david-hackam/necrotizing-enterocolitis/Mpv6w2_G/t5RhXw.

NEC pearls for the ED

TreatmentBroad spectrum antibiotics (Amp/Gent ± Flagyl)NPONG decompressionSerial X-rays Consult surgery

Lacy Gomella. Neonatology. 2004

Apnea of prematurity

Apnea of infancyUnexplained cessation in breathing > 20s or < 20s if

Bradycardia Cyanosis Pallor Hypotonia

Apnea of prematuritySame as above but < 37 weeks GA


Apnea of prematurity

More common with younger GA

Etiology is multifactorialCombination of central/obstructive apnea

Treatment in NICUCaffeine, though should be discontinued by

dischargeCPAP

AOP pearls in the ED

Typically, NICUs keep babies 8 days after last apnea episode

If truly AOP, should resolve by 43 weeks PCA

SIDS is not prolongation of apnea of prematurity

Apnea in someone who was previously discharged from the NICU a few days ago requires careful considerationAOP or not?ALTE?

Post-hemorrhagic Hydrocephalus

Results from intraventricular hemorrhage

Risk of IVH goes up with lower GAScreening protocols

Secondary to bleeding from germinal matrix in lateral ventricles

Agamanolis. Neuropathology. 2010. http://neuropathology-web.org/chapter3/chapter3dGmh.html

http://www.chop.edu/export/system/galleries/images/hospital/conditions/brain-tumors-161397.gif

VP Shunt Dysfunction/Infection

Symptoms = non specific but may include headache/vomiting/mental status/fever

Diagnosis = Push the valve (operation varies on the valve)CT/MRI head to rule out worsening ventriculomegalyShunt Series (Skull x-ray, CXR, AXR)Shunt Tap

TreatmentNeurosurgery consult for ± shunt revision

Take home points about prems

They come with lots of comorbid diseases

BPD, NEC, Apnea of Prematurity, VP Shunt Dysfunction are just a few

Parents often know more about their child’s conditions than you do

Objectives

Discuss the history of ALTEs, diagnostic work-up, and follow-up

Review risk factors for SIDS

Review conditions commonly encountered in the ED relevant to prematurely born patients

altes, sids, and prems russell lam september 1, 2011 special thanks to bela sztukowski for her help...

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