sudden infant death syndrome

1. Apparent Life Threatening
Event (ALTE(
Dr Mohamed I. AbunadaDr Mohamed I. Abunada
Pediatric NeurologyPediatric Neurology
Dr Alrantisi spezialisedDr Alrantisi spezialised
ped Hospitalped Hospital

2. Case
2 mo old female infant presents to ED via
ambulance after her mother witnessed an
episode of turning blue and having stopped
breathing. Mother tried stimulating baby
and after 1-2 minutes baby opened eyes.
Mother called 110 immediately.

3. Case Definition
 ALTE was defined in 1986 at the NIH Consensus
Development Conference on Infantile Apnea and Home
Monitoring as:
– “an episode that is frightening to the observer
and is characterized by some combination of
apnea, color change, marked change in
muscle tone, choking or gagging.”

4. Introduction
ALTE is a poorly defined term
ALTE infants represent an extremely
heterogeneous group of patients of varying
ages with diverse pathophysiology
Important to recognize that ALTE is NOT
a specific diagnosis; rather it describes a
symptom/chief complaint that brings the
infant to medical attention

5. Diagnostic Evaluation
HAVING A GOOD
DIFFERENTIAL DIAGNOSIS WILL
BE THE KEY!
Differential diagnosis of ALTE…

6. Differential Diagnosis
 Respiratory
 Laryngomalacia/
 tracheomalacia
 Breath holding spells
 Aspiration
 Central apnea
 Isolated prolonged apnea
 Vocal cord dysfunction
 Vascular ring
 Upper airway
obstruction
 Infectious
 Sepsis
 Bronchiolitis
 Pertussis
 Meningitis
 Pneumonia

7. Differential Diagnosis (cont(
 Neurologic
 Seizure
 Encephalopathy
 Structural abnormality
 Intracranial hemorrhage
 Increased ICP
 Gastrointestinal
 GERD
 Suck-swallow
incoordination

8. Differential Diagnosis (cont(
 Cardiac
 Congenital heart disease
 Dysrhythmia
 Cardiomyopathy
 Trauma
 Forced aspiration
 Suffocation
 Shaken baby
 Munchausen’s by Proxy

9. Differential Diagnosis (cont(
Metabolic
 Inborn error
 Electrolyte abnormality
 Unknown
 As many as 50% will
ultimately fall into
this category

10. History Taking – the event
 State – asleep, awake, crying, relationship to feeding or
emesis?
 Respiratory effort – none, shallow, increased, struggling, or
choking?
 Color – cyanotic, pale, gray, red ?
 Color change – entire body, extremities, face, peri-oral, lips?
 Tone – limp, rigid, tonic/clonic ?
 Eyes – open, closed, dazed, staring, rolled, bulging?
 Noise – none, cough, choking, stridor ?
 Fluid – none, mucus, milk, vomit, blood ?
 Duration – seconds, minutes?

11. History Taking - interventions
 None
 Gentle stimulation?
 Vigorous stimulation?
 Mouth to mouth resuscitation?
 CPR
 Duration?

12. History taking
 How was the baby behaving during the day prior to
the event?
 How did the baby act after the event?
 How long before the baby was acting at baseline,
and what was different until then?
 PH- focusing on detailed birth history, feeding
history, developmental history, h/o
tiring/cyanosis/sweating with feeds
 FH- focusing on cardiac hx, seizures, neuro
disease, genetic disease, SIDS, other ALTEs

13. Physical Exam
 General- Ill-appearing? Toxic? Arousable ? Tone?
 Fontanelle? Fundi ?
 Hemotympanum? Bleeding? Dysmorphic?
 Chest- Wheezing/crackles/cough? Tachypnea? Noisy
breathing/stridor?
 CV- Murmur? Pulses?
 Abd- Hepatomegaly?
 Ext- Bruising? Well-perfused?
 Neuro- Tone? Focal findings?

14. Initial Evaluation
 Admit orders include-
– Monitored bed
– CR monitor and continuous pulse ox
– Event log
 Remainder of management is based on likely
category of etiology as determined by initial H&P

15. Evaluation by Clinical Category
 Respiratory
 CXR
 ENT or Pulm eval for
direct airway visualization
 May overlap with
infectious and metabolic
 Infectious
 Respiratory viral screen
 Pertussis PCR
 CBC
 Blood/Urine Cx
 LP
 CXR

16. Evaluation by Clinical Category
 Gastrointestinal
 Reflux precautions
 Thickened feeds
 If severe apnea, consider:
 GI Consult
 Proton Pump Inhibitor
or H2 blocker
 Neurologic
 Seizure precautions
 Basic electrolytes,
glucose, calcium, Mg
 EEG
 Neuro consult
 Head imaging
 LP

17. Evaluation by Clinical Category
 Cardiac
 EKG
 CXR
 Echo
 Cardiology consult
 ABG/VBG
 Trauma.
 Child Protective Team
 Dilated retinal exam
 Head CT
 Skeletal survey
 Metabolic
 Electrolytes
 LFT
 Lactate, pyruvate
 Thyroid tests

18. Before Discharge
 Specific treatment will be dictated by the results of
the evaluation
 All parents receive CPR training
 SIDS prevention education

19. Home Apnea Monitors
 AAP Policy Statement. Pediatrics. 2003: 111: 914-917
 AAP Policy Statement. Pediatrics. 2005: 116:1245-
1255

20. SIDS Sudden Infant Death Syndrome
S – Sudden
I – Infant
D – Death
S – Syndrome

21. Definition of SIDS
Sudden Infant Death Syndrome (SIDS) is the
sudden death of an infant under one year of
age, which remains unexplained after a
thorough case investigation, including
performance of a complete autopsy,
examination of the death scene, and review of
the clinical history.
Source: Willinger M et al. Pediatr Pathol, 1991

23. Characteristics of SIDS
 Peak incidence at 2 to 4 months of age
 Slight male predominance
 More prevalent in cold, winter months
 Not considered genetic or hereditary
 Not due to suffocation, aspiration, abuse or
neglect
Source: CDC Facts About SIDS

24. Common Myths
To Dispel About SIDS
 SIDS is not caused by vaccines or immunizations
 SIDS is not contagious
 SIDS is not hereditary
 SIDS is not a result of child abuse or neglect
 SIDS, or “crib death”, is not caused by cribs
 Apnea monitors will not prevent SIDS
Source: NICHD Back To Sleep Campaign: Resource Kit for Reducing the Risk of SIDS in African American
Communities

25. What Causes SIDS?
SIDS
Exogenous
Stressors
Vulnerable
Infant
Critical
Development
Period
Researchers believe there is
probably more than one
cause of SIDS, and that
babies who die of SIDS are
born with abnormalities
which make them susceptible
to sudden death at critical
developmental periods. The
vulnerable infant is met with
an environmental challenge at
a critical developmental
period and cannot respond
adequately, resulting in
immediate death.
Source: Filiano JJ, Kinney HC. Biology of the Neonate, 1994
Triple-Risk Model

26. Five Steps in the Terminal Respiratory Pathway Associated with the Sudden Infant
Death Syndrome.
Death results from one or more failures in protective mechanisms against a life-
threatening event during sleep in the vulnerable infant during a critical period.

27. Risk Factors for SIDS
 Prone sleep position for
infants (stomach sleeping)
 Large, soft bulky blankets
 Large objects in sleeping
area
 Soft mattress
 Lack of (or late) prenatal
care
 Maternal age below 20
(adolescent mothers)
 Maternal substance use
during pregnancy
 Low birth weight,
premature and
multiple-birth infants
 Exposure to cigarette
smoke (prenatal and
postnatal)
 Low socioeconomic
status
Sources: NICHD, Sudden Infant Death Syndrome, April 1997. Sullivan FM, Barlow SM, Paediatr Perinat
Epidemiol, 2001

28. Key Points to Communicate
to Parents and Caregivers
 Always place baby on his/her back to sleep, even for
naps.
• Since 1994, it has been recommended that babies always be put to
sleep on their back.*
• This is the best way you can help reduce your baby’s risk for SIDS.
Even though the cause is unknown, it is known that infants put to
sleep on their back are less likely to die of SIDS.
• Even if bedsharing, make sure baby is sleeping face up.
*Source: AAP Task Force on Infant Sleep Position and SIDS, Pediatrics 2000
X
X

29.  Remove soft and fluffy bedding from sleep
area.
• This includes pillows, quilts, toys, and stuffed animals.
 Don’t allow the baby to sleep on soft
surfaces.
• This includes the couch, sofa, adult bed, waterbed or other
soft surfaces.
• Use a firm, tight-fitting mattress for the crib.
• Make sure baby can’t get trapped between the sofa cushions,
mattress or framework of the bed or crib.
Key Points to CommunicateKey Points to Communicate
to Parents and Caregiversto Parents and Caregivers

30. Key Points to Communicate
to Parents and Caregivers
 Make sure baby’s head and face remain
uncovered during sleep.
Keep baby’s mouth and nose clear of any blankets or
coverings.
Don’t let baby get too warm while sleeping.
Babies should not be tightly bundled or “swaddled” in a
blanket, even in hospital settings.
Remember that a baby’s environment should be the same
temperature that is comfortable for an adult, about 70° F.

31.  Create a smoke-free environment for your
baby.
• Don’t smoke during pregnancy or after the birth of your baby.
• Make sure that no one smokes around your baby.
Key Points to CommunicateKey Points to Communicate
to Parents and Caregiversto Parents and Caregivers

32. Common Barriers To “Back-To-Sleep”
 Grandparents and other caregivers
recommend putting baby to sleep on
their stomach
– This includes older caregivers as well as mothers who
gave birth to their last child more than 10 years ago.
– Ask them why they choose to use the prone position
and address how to overcome these concerns.
– Discuss how practices have changed over the years,
re-educate caregivers. “Did you know that we are
supposed to put babies to sleep on their backs?”
Source: NICHD Back To Sleep Campaign: Resource Kit for Reducing the Risk of SIDS in African American
Communities

33. Common Barriers To “Back-To-Sleep”
A belief that baby will develop a
permanent flat spot or bald spot on
his head
– Remind parents/caregivers that this is a
passing condition that goes away after baby
starts learning to sit up.
– This can be avoided with “tummy time” –
placing the baby on his stomach when he is
awake and is being observed.

34. A common belief that baby can choke in
the back-sleep position
– Some caregivers may need more intense education
to dispel this misconception, since this is often one
of their main reasons against using the supine
position.*
– Inform parents/caregivers that infants automatically
swallow or cough up fluid. The number of babies
dying on their backs has not increased since the
initiation of the Back-to-Sleep campaign in 1994.
Common Barriers To “Back-To-Sleep”
Source: NICHD Back To Sleep Campaign: Resource Kit for Reducing the Risk of SIDS in African American Communities,
* NYC DOHMH, Report on SIDS Focus Groups, 2004, unpublished data.

35. Common Barriers To “Back-To-SleepCommon Barriers To “Back-To-Sleep””
 A belief that babies sleep better on their
stomachs
 Most babies get used to the back position quickly. The
earlier you put baby on his/her back to sleep, the more
quickly baby gets used to it.
 Babies sleeping on their stomachs may not wake up as
quickly if something is wrong.
 Parents can comfort baby by putting her to sleep on
their chest, and then laying sleeping baby on her back
in the crib.

36. They put the baby to sleep on his
side or stomach in the hospital
Studies have found that back-sleeping is the
safest position for infants, even safer than side
sleeping.
Side sleeping increases babies’ risk of rolling to
the prone position.
Common Barriers To “Back-To-Sleep”Common Barriers To “Back-To-Sleep”
Source: NICHD Back To Sleep Campaign: Resource Kit for Reducing the Risk of SIDS in African American Communities

37. Breastfeeding and SIDS
• Although several retrospective studies have demonstrated a protective
effect of breastfeeding on SIDS, other analyses and prospective cohort
studies failed to find such an effect after adjustment for confounding
variables.*
• However, breastfeeding is known to provide many other health benefits
and should be practiced exclusively for at least the first 6 months.
*Source: AAP Task Force on Infant Sleep Position and SIDS, Pediatrics 2000
“According to AAP, evidence is insufficient to
recommend breastfeeding as a strategy to reduce
SIDS.”
– AAP Task Force on Infant Sleep Position and SIDS, Pediatrics 2000*

38. Bedsharing: A Controversial Issue
There are insufficient data to conclude that bedsharing under carefully
controlled conditions is clearly hazardous or clearly safe.
– AAP Task Force on Infant Sleep Position and SIDS, Pediatrics 2000
Risks1-3
Research has shown that bedsharing
may increase risk for SIDS in certain
situations. This includes bedsharing
with a parent who is:
• A smoker
• Under the influence of alcohol,
drugs, or medications that may
impair arousal
• Extremely tired or ill
• Obese
Benefits4-6
Some believe bedsharing may have certain
benefits, such as:
• Increased breastfeeding
• Enhanced closeness between parent
and baby
• Increased awareness of parent to
infant’s needs and arousals
• More infant arousals and less deep
sleep for infant
* Note: No epidemiologic studies have shown
that bedsharing has a protective effect from
SIDS.1
Sources: 1) AAP Task Force on Infant Sleep Position and SIDS, Pediatrics 2000 2) NICHD, Sudden Infant Death Syndrome, April
1997 3) Association of SIDS and Infant Mortality Programs, 2001 4) 2001 UNICEF UK Baby Friendly Initiative/FSID, July 2003 5)
Willinger M et al, Arch Pediatr Adolesc Med, 2003 6) Brenner RA et al, Arch Pediatr Adolesc Med, 2003.

39. “Safer” Bedsharing
 If Bedsharing, take care to observe the standard
recommendations for “safer” Bedsharing:
 Nonprone sleeping
 Avoidance of soft sleeping surfaces, loose adult
bedding (i.e. pillows, quilts, comforters), overheating or
overbundling.
 As an alternative to Bedsharing, parents may place baby’s
crib near their bed for more convenient breastfeeding and
parent contact.
Source: AAP Task Force on Infant Sleep Position and SIDS, Pediatrics 2000

40. Communicating with Patients
About Bedsharing
 Keep in mind that Bedsharing is a common practice in many
cultures and households.*
 If parents decide to share a bed with the baby, make sure to
discuss “safer” Bedsharing practices.
 Parents and caregivers should not bedshare if the adult is a
smoker, is very tired or ill, or is under the influence of
substances (such as alcohol, drugs, or prescription
medications) that may impair arousal.
* Sources: Willinger M et al. Arch Pediatr Adolesc Med, 2003; Brenner RA et al. Arch Pediatr Adolesc Med, 2003.

41. Scene Size Up
 DOCUMENT ALL DETAILS CAREFULLY
AND ACURATELY:
– Clothing, position of child, crib or bed, bedding,
objects in bed, any unusual odors, room
temperature, medications (OTC or other)
– DOCUMENTATION OF EVERY DETAIL
IS VERY IMPORTANT; ALL DEATHS
ARE CONSIDERED CRIME SCENES
UNTIL PROVEN OTHERWISE.

42. Event History
 Retrieval of a good history:
– What happened
– Infant location (who found infant)
– Interventions (CPR or other) by
parents/caregivers
– When was the infant last seen alive
– Recent illnesses and past medical history
– Any meds (OTC or others) the infant has received

43. Patient Assessment
 External Appearance with SIDS:
– Cold skin
– Frothy or blood tinged fluid in mouth and/or nose
– Normal hydration and nutrition
– Lividity and rigor mortis
– Vomitis is occasionally present
– ENSURE ALL FINDINGS AND
OBSERVATIONS ARE DOCUMENTED

44. SIDS prevention

45. Sleep Sacks
 To eliminate the possibility of loose blankets covering the infant's
face and to reduce the likelihood of overheating, the AAP
recommends using sleep clothing with no other covering or using
infant sleep sacks. Properly designed sleep sacks keep the
infant warm without the chance that the garment might cover the
head. In our NICU, infants were initially placed in sleep sacks
hand-sewn by a local church group that volunteered its services.
The sleep sacks were sized appropriately, had openings for
monitor wires, and were placed on all infants when they were
moved to an open crib. Parents could observe their infant in the
supine position wearing a safe sleep garment, which reinforces
SIDS prevention strategies.

46. Halo Sleep Sack wearable blanket

47.  A 10-year-old boy is brought to the emergency department after being
struck by a car while riding his bicycle. Physical examination
immediately upon arrival reveals that he is responsive to painful
stimuli only by moaning. His pupils are equal, small, and reactive.
Of the following, the sign MOST LIKELY to be associated with
increasing intracranial pressure in this patient is
A. hyperventilation
B. hypotension
C. hypothermia
D. nystagmus
E. tachycardia

48. A

49. You are seeing a 2-month-old male infant who has trisomy 21 for a health supervision visit. The
boy's mother expresses concern that the infant has been having "noisy breathing" during the
past 2 to 3 weeks. The infant has been exclusively formula-fed and has had no choking or
difficulty feeding. According to the mother, the noise, which occurs on inspiration, is louder
when the infant is supine and when crying.
She has not noticed any rhinorrhea, cough, or other upper respiratory viral illness symptoms.
The infant was born via an uneventful vaginal delivery that did not require forceps.
Apgar scores were 8 and 9 at 1 and 5 minutes, respectively.
On physical examination, the infant, whose physical appearance is consistent with trisomy 21,
is resting comfortably. His vital signs are appropriate for age, but you hear
an audible noise during inspiration.
Of the following, the MOST likely explanation for the infant's respiratory symptoms is
A. laryngomalacia
B. subglottic tracheal web
C. tracheomalacia
D. vascular ring
E. vocal cord paralysis

50. When evaluating an infant who presents with “noisy breathing,” it is important to obtain a
thorough birth history and current medical history, observe breathing patterns in different
positions, and auscultate the airway. Secondary evaluation tools include radiography,
spirometry, and direct airway visualization.
In general, breathing noises can be classified as inspiratory, expiratory, or biphasic.
Disorders at the level of and superior to the vocal cords result in a harsh, inspiratory noise
called stridor (Item C111A). Lesions inferior to the vocal cords typically result in wheezing, a
high-pitched expiratory noise.
Laryngomalacia is the most common congenital laryngeal abnormality resulting in stridor.
Symptoms may begin shortly after birth, although they typically occur between 1 and 2 months
after birth. As with the child described in the vignette, infants are happy, thriving, and not having
difficulty during feedings, but stridor usually worsens during supine positioning, increased crying
or agitation, or a viral illness. Direct visualization of an omega-shaped epiglottis that prolapses
(Item C111B) during inspiration is a hallmark of the condition. Severe cases may require surgical
correction, although laryngomalacia usually improves spontaneously by 2 years of age.
A laryngeal web results from failure of the embryonic airway to recanalize. Most laryngeal
webs occur at the level of the vocal cords and present at birth with stridor. The delayed onset of
stridor in the infant in the vignette makes a laryngeal web unlikely.
Tracheomalacia (Item C111C) results from defective cartilaginous rings causing flaccidity of
the tracheal wall. Both tracheomalacia and laryngomalacia present at similar ages and are
exacerbated by crying, viral respiratory infections, and supine positioning. However, infants who
have tracheomalacia present with wheezing instead of stridor.
Extrinsic compression of the trachea by vascular anomalies such as a vascular ring (Item
C111D) can result in recurrent wheezing that is worsened with crying, feeding, or neck flexion.

51. A 3-year-old boy presents to the emergency department following the
abrupt onset of Coughing and wheezing. You order a chest radiograph
for evaluation of a suspected foreign body aspiration.
Of the following, the MOST appropriate statement regarding foreign body aspiration i
s that
A. most foreign body aspirations present within 24 hours
B. nonfood items (eg, coins, pins, pencaps) are the most
common items aspirated by infants and toddlers
C. the classic triad of cough, wheeze, and decreased breath sounds
is present in most cases
D. the majority of aspirated foreign bodies are located in the larynx
or trachea
E. toy balloons are a common cause of foreign body aspirations

52. The presentation of a foreign body aspiration depends on whether the event was witnessed, the
age of the child, the type of object aspirated, the elapsed time since the event, and the location
of
the object. Most foreign body aspirations (50% to 75%) present and are diagnosed within 24
hours. Although an acute choking or coughing episode accompanied by wheezing is highly
suspicious for a foreign body aspiration, some infants and children present with few or no
symptoms. The classic triad of coughing, wheezing, and decreased breath sounds is present in
less than 50% of cases.
Food is the most common item aspirated by infants and toddlers (eg, sunflower seeds, nuts,
beans, carrots, corn); nonfood items (eg, coins, paper clips, pins, pen caps) more commonly
are aspirated by older children. Although a rare cause of foreign body aspiration, toy balloon
aspiration can be fatal.
Most aspirated foreign bodies lodge in the right lung rather than the larynx or trachea (). Long-
term complications of undiagnosed or retained foreign bodies include recurrent
pneumonia, atelectasis, and bronchiectasis.
A

53.  All of the following statements concerning acute
epiglottitis are correct, EXCEPT:
A) it progresses slowly
B) fever and excitement are observed
C) dyspnea, cyanosis and retroflection of the head
are observed
D) suffocation might develop

54. A

55. Which of the following statements about subglottic
laryngitis is correct?
A) it is most commonly manifested between the
ages of 8-10y
B) it is always associated with a high fever
C) the speech is clear
D) an expiratory type dyspnea develops
E) a "barking" cough is detected

56. E

57. Which of the following procedures is reliable in
the diagnosis
or exclusion of a foreign body in the airways?
A) a physical examination
B) a thorough history taking
C) bronchoscopy
D) chest transillumination (Holzknecht's sign)
E) chest x-ray

58. C