ALTEs , SIDS, and Prems

ALTEs, SIDS, and Prems

Russell LamSeptember 1, 2011

Special thanks to Bela Sztukowski for her help on this presentation

ObjectivesDiscuss the history of ALTEs, diagnostic work-up,

and follow-upReview risk factors for SIDSReview 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

DefinitionsApparent Life Threatening Event

Frightening 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.

DefinitionsSudden infant death syndrome

Death 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

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

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

DefinitionsApnea of infancy

Unexplained cessation in breathing > 20s or < 20s if Bradycardia Cyanosis Pallor Hypotonia

Apnea of prematuritySame as above but < 37 weeks GA

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

Who gets ALTEs?0.5-6% of all infantsDifficult 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 diagnoses

GERD (31%)Seizure (11%)LRTI (8%)Unknown (23%)

Serious Bacterial Infection?Altman (2008) – Retrospective chart review N=243

5% had occult bacterial infection26% had obvious bacterial infection

Mittal (2009) – Prospective cohort N=19822.2% had cultures0% had serious bacterial infection

Zuckerbraun (2009) – Retrospective chart review N=18261.5% had cultures2.7% had serious bacterial infectionPremature 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 = 598 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 ALTEsFrom 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 = 5633 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 ALTEScary+ Apnea/Colour Change/Tone/ChokingBroad differential but mostly GER/LRTI/CNSLikely need admission + broad work-upLow mortality rate (0.5%)ALTE ≠ SIDS

Case 26 mo male brought in because of cough x 3 daysYou diagnose URTI and discharge the patientOn 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 SIDSMost common cause of death in 1mos-1y (20-25%

of all deaths < 1 year)2006 = 0.54 per 1000 live births in the USMost will occurs age 2-4 months, almost all by 6

months

SIDS versus SUDISudden 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 proneMaternal smoking during and after pregnancyBed-sharing, especially if EtOH or very tired

parentSoft bedding, pillow, covers over the headPrematurity (<37 weeks)Low birth weight (<2500g)

A safe sleeping environmentCPS 2004 Guidelines

1st six months babies should sleep in own crib in parent’s room

Sleep 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 2005

Many 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 deaths

RR 1.13 (95%CI 0.97-1.31) for twins when adjusted for birth weight

RR 8.17 (90%CI 1.18-56.67) if 1 twin died of SIDSGetahun (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 SIDSDifferent from ALTETwo most important risk factors are prone sleeping

and maternal smokingBack to sleep in their own cribDon’t discourage pacifiersApnea monitors don’t helpIf a twin already died of SIDS, other twin at way

higher risk. Admission debatable…

Case 32 month old ex 24 week male comes in with wheeze

and coughMom 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 LingoPrematurity = <37 weeks gestational ageBirth weight

Low birth weight = < 2500gVery low birth weight = < 1500gExtremely low birth weight = < 1000g

The Lingo

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

Bronchopulmonary DysplasiaDefined by oxygen needs beyond 28 days of lifeInitial 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 EDExamines like asthma

Increased RR, wheeze, cracklesTreat like asthma

SABA, Inhaled Corticosteroids, Oxygen

Consider diureticsSpecial 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 EnterocolitisSpectrum of acquired neonatal disease with end

expression of serious intestinal injuryEtiology 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 asSeptic infantLower GI BleedingAbdominal 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.

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 EDTreatment

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

Lacy Gomella. Neonatology. 2004

Apnea of prematurityApnea of infancy

Unexplained cessation in breathing > 20s or < 20s if Bradycardia Cyanosis Pallor Hypotonia

Apnea of prematuritySame as above but < 37 weeks GA

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

Apnea of prematurityMore common with younger GAEtiology is multifactorial

Combination of central/obstructive apnea

Treatment in NICUCaffeine, though should be discontinued by

dischargeCPAP

AOP pearls in the EDTypically, NICUs keep babies 8 days after last

apnea episodeIf truly AOP, should resolve by 43 weeks PCASIDS is not prolongation of apnea of prematurityApnea in someone who was previously discharged

from the NICU a few days ago requires careful considerationAOP or not?ALTE?

Post-hemorrhagic HydrocephalusResults from intraventricular hemorrhageRisk of IVH goes up with lower GA

Screening protocols

Secondary to bleeding from germinal matrix in lateral ventricles

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

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

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

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/InfectionSymptoms = non specific but may include

headache/vomiting/mental status/feverDiagnosis =

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 premsThey come with lots of comorbid diseasesBPD, NEC, Apnea of Prematurity, VP Shunt

Dysfunction are just a fewParents often know more about their child’s

conditions than you do

ObjectivesDiscuss the history of ALTEs, diagnostic work-up,

and follow-upReview risk factors for SIDSReview conditions commonly encountered in the

ED relevant to prematurely born patients