Sudden Infant Death Syndrome (SIDS), Emergency Medicine

Basics

Description

Sudden, unexpected death of an infant <1 yr old who was typically well before being placed down to sleep
Death remains unexplained after being thoroughly investigated by autopsy, exam of the death scene, investigation of the circumstances, and review of the family and infant medical histories.
Leading cause of death in infants 1 mo " “1 yr of age; the incidence has declined markedly since the initiation of the "Back to Sleep " ť program in 1994:
- 1992: 120 deaths/100,000 live births (US)
- 2001: 56 death/100,000 live births (US)
- No change from 2001 " “2006
Peak occurrence of SIDS at 1 " “4 mo of age:
- 90% occur <6 mo of age
- 2% occur >10 mo of age
Ethnic differences: 2006 rates per 100,000 live births: All populations, 54.5; non-Hispanic white, 55.6; non-Hispanic black, 103.8; American Indian/Alaska Natives, 119.4; Asian American or Pacific Islander, 22.8; Hispanic, 27.
Sleeping on back (supine) reduces incidence significantly ( "Back to Sleep " ť). Practice of infants sleeping on their backs began initially in Europe and then in US

Etiology

Most likely multifactorial
SIDS infants likely have predisposing conditions that make them more vulnerable to both internal and external stressors.
Potential stressors include anemia, congenital diseases, dysrhythmias, electrolyte abnormalities, genetic defects, infection, metabolic disorders, neurologic events, suffocation, trauma, upper airway obstruction.
Maternal and antenatal risk factors:
- Alcohol and illicit drug use
- Intrauterine growth restriction
- Lower socioeconomic status
- Poor prenatal care
- Prior sibling death secondary to SIDS
- Shorter interval between pregnancies
- Smoking
- Younger age
Infant risk factors:
- Bed sharing
- Exposure to environmental smoking
- Gastroesophageal reflux (GER)
- Hyperthermia
- Low birth weight, prematurity
- Male gender
- Soft bedding, soft sleeping surface
- Recent febrile illness
Supine sleeping position, breast-feeding, and pacifier use are protective.
Home monitoring has not been shown to prevent SIDS.

Diagnosis

Signs and Symptoms

History

No significant pre-existing signs or symptoms to alert caretakers
Unpredictable
Most infants appear normal when put to bed.
Death occurs while the infant is sleeping.
Typically the event is silent with no signs of struggling.
No clinical or pathologic explanation for death.
Apparent life-threatening event (ALTE) is an acute event that is frightening to the caretaker:
- Characterized by apnea (either central or obstructive) causing changes in skin color " ”cyanosis, pallor, or erythema with limpness, choking, and/or gagging.
- Infant should be transported to hospital for evaluation and monitoring.
- Appears well when evaluated by clinicians after recovery from ALTE.
- Associated with an increased risk of SIDS.

Physical Exam

Prior to the event, the infant is seemingly healthy and well appearing, well developed, and well nourished.
If event was brief and self-limited, may appear well when evaluated after the episode.
Potential complications for surviving infants include pulmonary edema, aspiration pneumonia, and neurologic sequelae secondary to hypoxia including seizures.

Essential Workup

SIDS is a diagnosis of exclusion, so requires an evaluation to identify primary and/or contributing conditions.
Thorough investigation of the death scene:
- Conditions surrounding sleeping space (temperature, surface, bedding, bed sharing)
- Position in which infant was sleeping
- Interview of parents, family members, and caregivers
- Exam of potentially relevant items from the death scene
- Maintain sensitivity toward family as investigation may be difficult for them.
Review infant and family histories:
- Prenatal, perinatal, and postnatal infant medical history
- Family medical and social histories, particularly mother
Impact of investigation on family:
- Family is very vulnerable during the investigation
- May help them through the grieving process.

Diagnosis Tests & Interpretation

Lab

Selective studies reflecting nature of episode and patient condition
Arterial/venous blood gas
Blood culture and other sepsis workup as indicated
CBC
ECG
Include family member to evaluate for familial dysrhythmias such as prolonged QT syndrome
EEG
Electrolytes including calcium, magnesium and phosphorous. Liver function tests
Toxicology screen
UA and culture

Imaging

Chest radiograph to assess cardiopulmonary status
Skeletal survey to evaluate for child abuse (may be performed by pathologist)
Head CT if child survives to assess intracranial pathology
Consider upper GI to evaluate for GER

Diagnostic Procedures/Surgery

Autopsy:
- Most states require an autopsy for potential SIDS cases
- Important that postmortem exam be performed as SIDS is a diagnosis of exclusion
- Involves microscopic exam of vital organs through tissue samples as well as gross exam
- Some postmortem findings in SIDS cases that might establish alternative cause of death:
  - Congenital cardiomyopathies
  - Cardiac rhabdomyomas
  - Tuberous sclerosis
  - Rare genetic/metabolic diseases
  - Viral myocarditis
  - Intracranial arteriovenous malformations

Differential Diagnosis

Cardiovascular:
- Anomalous coronary artery
- Aortic stenosis
- Cardiomyopathy
- Dysrhythmia
- Myocarditis
Respiratory:
- Aspiration
- Suffocation
Infection:
- Botulism
- Bronchiolitis/respiratory syncytial virus
- Encephalitis
- Meningitis
- Pertussis
- Sepsis
CNS:
- Arteriovenous malformation
- Central hypoventilation
- Neuromuscular disorders
- Seizures
- Tuberous sclerosis
GI:
- GER
- Diarrhea
- Pancreatitis
- Volvulus
Endocrine/metabolic:
- Carnitine deficiency
- Congenital adrenal hyperplasia
- Glycogen storage disease
- Long- or medium-chain acyl-coenzyme A deficiency
- Urea cycle defect
Systemic:
- Child abuse
- Dehydration
- Intentional poisoning
- Hyperthermia

Treatment

Initiate resuscitation at the scene; transport infant to ED and continue protocols en route.
On very rare occasion and under medical direction, resuscitations have been aborted and the infant is pronounced at the scene; consideration must be given to the emotional, social, and clinical circumstances.

Pre-Hospital

Resuscitation procedures supplemented by support for the family
Evaluate setting; determine if suspicion of abuse

Initial Stabilization/Therapy

Assess and support ABCs (bedside).
Administer appropriate medications per protocols by endotracheal tube if IV access unobtainable (atropine, epinephrine, lidocaine, and naloxone).
Monitor vital signs: BP, heart rate, respirations, and oxygen saturation continuously.
Conduct a thorough physical exam; look for unintentional as well as intentional traumas.
Assess the scene, family members, and other caretakers.

Ed Treatment/Procedures

Resuscitate patient per established protocols continuing efforts initiated by pre-hospital personnel:
- Health care providers are encouraged to offer family members the opportunity to be present during resuscitation.
If resuscitation unsuccessful and no obvious diagnosis found, parents should not be told that SIDS is the cause of death:
- In speaking with the parents, SIDS may be included among the possible causes of death.
- A diagnosis cannot be made until completion of an autopsy, investigation of circumstances and death scene, and exploration of the medical histories of the infant and family.
Family support:
- If resuscitation unsuccessful, attention should then focus on the family; if resuscitation ongoing, communication and support of family is essential.
- All family members and caregivers are affected; they experience grief, guilt, failure, and inadequacy.
- Some parents want to spend quiet time holding their infants after an unsuccessful resuscitation.
- Family is defined variably among different cultures; ED personnel should attempt to be sensitive to cultural needs and expectations of the family.
- Family should be offered support in the ED and supplied with resources of support for beyond the day of the infants death; local, state, and national SIDS Foundation resources should be made available.
- Support may be obtained from Sudden Infant Death Syndrome Alliance/First Candle, 1314 Bedford Avenue, Suite 210, Baltimore, MD 21208 (800-221-7437) or local SIDS support organization.
- The child's PCP should be involved in the follow-up and support of the family.
Emergency personnel support:
- ED debriefing should be conducted for all staff who were involved in the infant's care, including EMS personnel; it is important to allow people to express their feelings and freely process the event in a supportive environment.

Follow-Up

Disposition

Admission Criteria

Observe all infants who have ALTE for evaluation and monitoring after initial resuscitation and stabilization.
Most high-risk infants have 1 of the following variables: Obvious need for admission, significant medical history, and >1 ALTE in 24 hr.

Discharge Criteria
Patients are generally admitted for observation and monitoring for documented episodes and support of family. ‚
Issues for Referral

All surviving infants should have a pediatric consultation.
Families will need support.

Pearls and Pitfalls

Infants with SIDS or ALTE should be resuscitated appropriately. Autopsies are essential for diagnosis and should be considered mandatory. Use available resources including social workers and chaplains as support for the family is crucial. ‚

Additional Reading

Carroll-Pankhurst ‚ C, Mortimer ‚ EA Jr. Sudden infant death syndrome, bed sharing, parental weight, and age at death. Pediatrics. 2001;108:1239 " “1240.
Fu ‚ LY, Colson ‚ ER, Corwin ‚ MJ, et al. Infant sleep location: Associated material and infant characteristics with sudden infant death syndrome prevention recommendations. J Pediatr. 2008;153:503 " “508.
Hauck ‚ FR, Omojokun ‚ OO, Siadaty ‚ MS. Do pacifiers reduce the risk of sudden infant death syndrome? A meta-analysis. Pediatrics. 2005;116:e716 " “e723.
Kaji ‚ AH, Claudius ‚ I, Santillanes ‚ G, et al. Apparent life-threatening event: Multicenter prospective cohort study to develop a clinical decision rule for admission to the hospital. Ann Emerg Med. 2013;61:379 " “387.
Krous ‚ HF, Beckwith ‚ JB, Byard ‚ RW, et al. Sudden infant death syndrome and unclassified sudden infant deaths: A definitional and diagnostic approach. Pediatrics. 2004;114:234 " “238.
Paris ‚ CA, Remler ‚ R, Daling ‚ JR. Risk factors for sudden infant death syndrome: Changes associated with sleep position recommendations. J Pediatr. 2001;139:771 " “777.
Task Force on Sudden Infant Death Syndrome, Moon ‚ RY. SIDS and other sleep-related infant deaths: Expansion of recommendations for a safe infant sleeping environment. Pediatrics. 2011;128:e1341 " “e1367.

Codes

ICD9

798.0 Sudden infant death syndrome ‚

ICD10

R99 Ill-defined and unknown cause of mortality ‚

SNOMED

51178009 Sudden infant death syndrome (finding)

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