Prematurity neonatology sids
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Prematurity, Neonatology, SIDS. Jay Green Emergency Medicine Resident, PGY-2 July 19, 2007. Outline. Apparent Life-Threatening Events Sudden Infant Death Syndrome Other causes of apnea ± Quick snappers Won’t cover Fever/sepsis in the newborn Bronchopulmonary dysplasia Cerebral palsy

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Prematurity neonatology sids

Prematurity, Neonatology, SIDS

Jay Green

Emergency Medicine Resident, PGY-2

July 19, 2007


Outline

Outline

  • Apparent Life-Threatening Events

  • Sudden Infant Death Syndrome

  • Other causes of apnea

  • ±Quick snappers

  • Won’t cover

    • Fever/sepsis in the newborn

    • Bronchopulmonary dysplasia

    • Cerebral palsy

    • Obstructive hydrocehpalus


Case 1

Case 1

  • 5mo M, stopped breathing x ?1-2min

  • Blue colour, limp

  • Resolved before EMS arrived

  • No vomiting, no sz activity

  • Position - supine

  • Noise - ?choking

  • No abnormal eye mvts

  • No intervention by parents


Case 1 cont

Case 1 cont

  • OB Hx: no complications, SVD @ 38wks

  • PMH: well child

  • FHx

    • øApnea, øSIDS, øSz, øCHD


Case 1 cont1

Case 1 cont

  • O/E:

    • Well-looking child

    • Vitals

      • HR 125, bp 85/55, RR 35, T 369

    • Nothing remarkable to find

  • Anything specific not to miss O/E?

    • Fundoscopy, SpO2


Prematurity neonatology sids

  • What is on your differential diagnosis?


Apparent life threatening event

Apparent Life-Threatening Event

ALTE


Alte definition

ALTE Definition

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

    • Apnea

    • Colour change

    • Marked change in muscle tone

    • Choking

    • Gagging

      National Institutes of Health Consensus Development

      Conference on Infantile Apnea and Home Monitoring


Alte quick stats

ALTE Quick Stats

  • Incidence 0.5-6%

  • 4-8% of SIDS had a previous ALTE

    • Not considered same disease process

  • 82% occur between 8am-8pm

  • Usually < 6mo, avg 8-14wks

  • Can be > 1yr

  • 13% risk of death if needed CPR and discovered during sleep


Alte hx exam

ALTE Hx/Exam

  • Most NB parts of ED diagnostic evaluation

  • History

    • Colour, tone, resp effort

    • Onset (sleep, feeding, awake), duration

    • Position (prone, sitting, supine)

    • Noises (stridor, choking)

    • Eye movements

    • Vomiting

    • Intervention


Alte exam

ALTE - Exam

  • PE usually normal

  • N = 73

  • Dilated fundoscopic exam

    • Retinal hemorrhages in 1pt, child abuse in 4


Back to case 1

Back to Case 1

  • 5mo M ?ALTE

  • What would you like to do now?

    • Labs?

    • Imaging?

    • Discharge patient?


Alte investigations

ALTE Investigations

  • 50% have specific diagnosis found

    • Infection, GI, Sz


Alte investigations1

ALTE Investigations

  • 196 infants with ALTE, mean age 2mo

  • 83% hospital admission

  • 50% had normal exam

  • 25% had infection/fever

  • Diagnoses:

    • Seizure (25%), GER (18%), febrile convulsion (12%), LRTI (9%), apnea (9%)

  • No infant subsequently died


Prematurity neonatology sids

  • 65 infants with ALTE, mean age 7wks

  • 100% hospital admission (required)

  • 54% had normal exam

  • Diagnoses:

    • GER (25%), unknown (23%), pertussis (9%), Other LRTI (9%), Sz (9%), UTI (8%)

  • No infant subsequently died

Thanks Yael!


Prematurity neonatology sids

  • Investigation protocol

    • 13% anemia, 33% ↑WBC (50% had inf)

    • Metabolic screen, urine reducing substances, ammonia not helpful

    • ↓Bicarb in 20% - 7 dx with sepsis/sz

    • ↑Lactate in 7, 5 had serious illness

    • U/A, pertussis swab useful in 5% & 8%

    • CXR abN in 9 who had N exam


Return to case 1

Return to Case 1

  • Labs N

  • CXR N

  • ECG N

  • Nasal swab, urine cultures pending

  • What would you like to do now?


Alte some perspective

ALTE - Some Perspective

  • Pre-hospital study, retrospective

  • N = 60, mean age 3.1mo

  • 83% no distress, 13% mild distress, 3% moderate distress

  • Diagnoses

    • Pneumonia (12%), sz (8%), sepsis (7%), ICH (3%), bacterial meningitis (2%), anemia (2%)

  • ALTE can be presenting sign of serious illness, even in well-looking child

Thanks Yael!


Alte disposition

ALTE Disposition

  • Most studies recommend mandatory period of inpatient observation

  • Majority suffer only 1 event

  • No single test has a high PPV for detecting anything that will alter the outcome

  • Recurrence rate for severe ALTE as high as 68% in one study

    • More likely in the few days after first event


Alte disposition1

ALTE Disposition

  • If no cause for ALTE found

    • Referred to as “apnea of infancy”

    • ±home apnea-bradycardia monitoring

      • Lack efficacy, frequent false alarms, misinterpretation of alarm by parents

      • Potential candidates

        • Premature infants exhibiting apnea beyond term

        • Term infants with ALTE requiring resus

        • Siblings of 2+ SIDS victims

        • Infants with BPD/tracheostomies


Alte causes

ALTE Causes

  • Infection

  • Seizure

  • A/W Obstruction

  • Breath-Holding Spells

  • GER

  • Metabolic

  • Nonaccidental

See EM Reports Aug 7, 2006


Alte sids

ALTE  SIDS?

  • Prospective cohort study, N=141, 8yrs

  • ?Association between SIDS & ALTE

  • Conclusions

    • RF for all ALTE’s

      • Common to SIDS: single parent, FHx infant death, smoking during preg, marked night sweating

      • Early behaviours: repeated apnea, cyanotic episodes, feeding difficulties, marked pallor

    • RF for “idiopathic ALTE”

      • No common SIDS RF

    • No subsequent SIDS deaths


Prematurity neonatology sids

  • Conclusions

    • ALTE/SIDS not part of the same disease process

    • SIDS prevention programs not expected to lower ALTE frequency


Alte take home points

ALTE Take-home Points

  • Scary + apnea, ∆colour, choking, ∆tone

  • Usually < 6mo

  • Well-looking ALTE  ?serious illness

  • Inpatient work-up

  • Not same disease process as SIDS

  • Questions?


Case 2

Case 2

  • 4mo F, found blue, not breathing in crib

  • EMS called, begin CPR, and patch in

    • Baby cyanotic, initial rhythm asystole, no resp efforts

  • What do you tell them?

    • Continue CPR and come in?

    • Call it in the field?


Sudden infant death syndrome

Sudden Infant Death Syndrome

  • Sudden death of an infant <1y old

  • Remains unexplained after investigation:

    • Complete autopsy

    • Examination of the death scene

    • A review of the clinical history

      National Institute of Child Health and Human Development


Sids fast facts

SIDS Fast Facts

  • US data

    • 0.72/1000 live births in 1998

    • Declining incidence

    • 3000 deaths/yr

  • 95% < 6-8mo, peak 2-4mo

  • 1% < 1mo, 2% > 2yr


Prematurity neonatology sids

  • What are some risk factors for SIDS?

  • Maternal

    • Smoking

    • Drug use

    • ↓SES

    • Age<20 at G1

    • Ethnicity

    • ↓Education

    • No prenatal care

  • Prenatal

    • IUGR

    • Multiples

    • Prematuriy

    • BW < 2500g

  • Postnatal

    • Prone sleeping

    • ETS

    • Warm temp

    • Loose bedding

    • Soft surface

    • Bed sharing

    • ?infection

    • ?GER

    • ?arrhythmia

What is the most important modifiable risk factor?

Prone sleeping 78%17%, SIDS ↓ 40%!


Sids what happens

SIDS – What Happens?

  • >70 theories: “triple-risk theory” – Rosen’s

Immature cardiorespiratory control

Autonomic dysfunction

Physiologic stuff

Predisposing factors

↓ baroreceptor reflex

↓vasomotor control

↓central venous return, CO, bp

Is sleep ever bad…I guess so…

Sleep

Exacerbate these effects

Progressive bradycardia

Poor lung perfusion  hypoxia

Various badness that doesn’t help

Prone sleep

URTI

Overheating

SIDS

SIDS


Case 2 cont

Case 2 cont

  • 4mo F just arrived in your ED

  • CPR continuing

  • Pupils fixed mid-dilated

  • Rhythm asystole

  • Unknown downtime

  • How long do you continue the resus?

    • ~3 rounds of drugs


Sids outcome

SIDS Outcome

  • After infant declared dead

    • Blood, urine, skin samples

    • Family meeting

    • Coroner notified

      • House inspection

      • Autopsy


Sids pathologically speaking

SIDS Pathologically Speaking

  • Nothing pathognomonic

  • Some typical findings

    • PA smooth muscle hypertrophy

    • RVH

    • ↑ hepatic hematopoiesis

    • ↑ periadrenal brown fat

    • Adrenal medullary hyperplasia

    • Carotid body abnormalities

    • Brainstem gliosis


Sids effects

SIDS Effects

  • Guilt, blaming, social alienation

  • ↑ miscarriage rate, divorce, infertility

  • Potentially helpful steps:

    • Openly accepting grief reactions

    • Allowing family to vocalize their feelings

    • Clarifying misconceptions

    • Allowing the family to hold/be along with infant

    • Private place for family to gather

    • Explanation of cause of death


Case 2 cont1

Case 2 cont

  • Unsuccessful resuscitation

  • Infant declared dead

  • Parents inform you that infant has a twin brother

  • What should you do about this?

    • Inform them there’s no increased risk?

    • Admit the twin for observation?


Sids twins

SIDS - Twins

  • Cohort studies looking at twins

    • Variable findings, 2x increased risk of SIDS

  • Any sibling of SIDS victims

    • 5-6x increased risk of SIDS

  • Reasonable to admit the twin for a period of observation


Sids prevention

SIDS Prevention

  • Non-prone sleeping (supine preferred)

  • No sleeping in waterbeds, sofas, soft mattresses/surfaces

  • No soft materials in sleeping env’t

  • Avoid bed-sharing and co-sleeping

  • Avoid overheating


Prematurity neonatology sids

  • Retrospective review, 10yrs

  • All deaths < 1yr in Quebec

  • 396 SIDS deaths

  • Infants <1mo

    • 10.2% died sitting

  • Infants >1mo

    • 1.4% died sitting

    • P<0.001

    • RR 7.35

  • ?↑ risk with ↑ time

  • ?↑ risk with position

  • No ↑ risk with premature infants

Conclusions:

-an excess of infants <1mo died

in sitting position compared to

those >1mo

-length of time in seat and

position may be NB contributors


Sids take home points

SIDS Take-home points

  • Peak age 2-4mo

  • Prone sleeping most NB modifiable RF

  • SIDS death can be called in the field

  • Resus of asystolic neonate x ~3 rounds

  • Admit twin of SIDS victim

  • Questions?


Apnea definitions

Apnea Definitions

  • Pathological apnea

    • Respiratory pause > 20sec or assoc with cyanosis, pallor, hypotonia, bradycardia

  • Apnea of prematurity

    • Periodic breathing with pathological apnea

  • Apnea of infancy

    • Infant > 37wks, pathological apnea or shorter apneic pauses & bradycardia, cyanosis, pallor, or hypotonia

    • “Idiopathic ALTE”


Case 3

Case 3

  • 10d F breathing pauses lasting ~5s

  • 4-5 episodes/min, comes & goes

  • Born at 39wks

  • Uncomplicated preg/delivery to G1P1

  • No fever, rash, lethargy

  • Feeding well

  • 10-12 wet diapers/d, 3-4 seedy stools/d

  • Regained birthweight at 7d


Case 31

Case 3

  • O/E

    • VS N

    • Well looking child, no apneic episodes in ED

  • What next?

    • Labs?

    • Imaging?

    • Discharge?

    • What do you think is going on?


Periodic breathing

Periodic Breathing

  • Normal

  • 3 or more pauses of >3sec with less than 20sec of N respirations between pauses

  • Treatment?

    • Caffeine


Methylxanthines

Methylxanthines

  • Helpful in apnea of prematurity and in reducing periodic breathing

  • Caffeine better than theophylline

    • Longer half-life

    • Wider therapeutic index

    • More reliable absorption

  • Caffeine citrate 20mg/kg IV/PO load

    • 5-8mg/kg OD

  • Why do we use caffeine?


Caffeine mechanism of action

Caffeine – Mechanism of Action

  • Increases levels of 3’5’-cyclic AMP by inhibiting phosphodiesterase

  • CNS stimulant

    • Increases medullary resp center sensitivity to CO2

  • Stimulates central inspiratory drive

  • Improves skeletal muscle contraction

    • Diaphragmatic contractility

  • Prevention of apnea may occur by competitive inhibition of adenosine


Caffeine

Caffeine

  • N=15 with periodic breathing (PB)

  • Conclusions

    • Weak correlation btw GER and PB

    • Theophylline/caffeine

      • Marked reduction of PB

      • Increases GER

Skopnik H et al.Effect of methylxanthines on periodic respiration and acid gastro-esophageal reflux in newborn infants. Monatsschrift Kinderheilkunde 1990;138(3):123-7


Case 4

Case 4

  • 4d M apneic episodes today lasting ~30s

    • ?A bit blue during episodes

  • Discharged from hospital today

  • Infant born @ 361 wks

  • Uncomplicated preg/delivery

  • O/E

    • VS N, well child, no apneic episodes in ED

  • Investigations?

  • Disposition?

  • What does this child have?


Apnea of prematurity

Apnea of Prematurity

  • Periodic breathing with apneic episodes > 20sec

  • Usually resolves by 37wks gestation

  • Management?

    • Inpatient work-up/monitoring

    • Caffeine citrate 20mg/kg IV/PO load

      • 5-8mg/kg OD


Apnea take home points

Apnea Take-home Points

  • Periodic breathing is normal

    • 3+ pauses >3sec with <20sec of N resps btw

  • Caffeine helps in periodic breathing and apnea of prematurity

  • Pathological apnea is >20sec

  • Pathological apnea always deserves W/U

  • Questions?


Quick snapper 1

Quick Snapper #1

  • 5d M poor feeding & vomiting x 1d

  • D/C yesterday

  • Born 361, difficult labour, decels, forceps

  • Breast-fed, with bottle supplementation

  • Gaining weight x 2d

  • No bloody stools, non-bilious emesis, no fever

  • O/E

    • Vitals N

    • Abdo ?distended

  • Investigations?


Quick snapper 11

Quick Snapper #1


Necrotizing enterocolitis nec

Necrotizing Enterocolitis (NEC)

  • Mucosal/transmural intestinal necrosis

  • Most common GI emergency, but often presents prior to d/c

  • 90% premature

  • >32wks usually present in 1st week of life

    • Can be >3mo in VLBW infants


Nec pathogenesis

NEC Pathogenesis

  • Unknown

  • Probably combination of

    • Mucosal injury (ischemia, infection, inflammation)

    • Host's response to injury (circulatory, immunologic, inflammatory)

  • RF

    • Aggressive enteral feeding, birth-related hypoxic-ischemic insults, infection


Prematurity neonatology sids

NEC

  • Radiological appearance

    • Dilated loops

    • Pneumatosis intestinalis (present in 75%)

    • Biliary tract air

    • Pneumatosis gastralis

    • Free air (only present in 50-75% with perf)

  • Labs not diagnostic

  • Treatment?


Nec management

NEC Management

  • Consult peds surgery

  • Admission

  • NPO

  • NG/OG

  • Careful fluid/lyte mgmt (3rd spacing)

  • ±Abx (amp/gent/flagyl)


Nec take home points

NEC Take-home Points

  • 90% are premature

  • Usually early but can be >3mo in VLBW

  • Pneumatosis intestinalis specific for NEC

  • Admit, NPO, Fluids, NG, ±Abx, ±Surgery

  • Questions?


Quick snapper 2

Quick Snapper #2

  • 6d F “off-colour” x 1-2 days - ?jaundice

  • Born 386, uncomplicated delivery via C/S

  • Feeding well, 10 wet diapers, 3 stool/d

  • Wt – regained birth weight today

  • No fever, lethargy, irritability

  • FHx: nothing metabolic/congenital

  • O/E

    • Well-looking child, VS N

    • Slight jaundice

  • Investigations?


Quick snapper 21

Quick Snapper #2

  • CBC N

  • Total bili = 200μmol/L

  • Conjugated bili not elevated

  • U/A –ve

  • What now?


Jaundice

Jaundice

  • Indications for further work-up?

    • Jaundice appearing <24h after birth

    • Elevated conjugated bili

    • Rapidly rising total serum bilirubin

    • Total serum bilirubin approaching exchange level or not responding to phototherapy

    • Jaundice persisting beyond age 3 weeks

    • Sick-appearing infant

Rosen’s


Neonatal jaundice

Neonatal Jaundice

  • HUGE differential

  • What does this infant have?

  • Physiological jaundice

    • 60% incidence 1st week of life

    • Gradual bili increase until 3rd day of life

    • Bili returns to N ~2wks

    • Why does this happen?


Quick snapper 22

Quick Snapper #2

  • 6d F

  • Jaundice, otherwise well-looking

  • Bili 200

  • Urine -ve

  • ?Physiologic jaundice

  • Management?


Neonatal jaundice management

Neonatal Jaundice Management

  • Continue breastfeeding

  • Monitoring

    • Homecare, FP

  • ±Phototherapy

  • ±Exchange transfusions

  • Complications?

    • Neurotoxicity, encephalopathy, kernicterus


Neonatal jaundice take home points

Neonatal Jaundice Take-home Points

  • 60% will get physiologic jaundice

  • Conjugated hyperbili is pathological

  • Jaundice in first 24h of life is pathological

  • Know indications for further W/U


The end

The End

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