Author:
KristineKnuti Rodrigues
Genie E.Roosevelt
Description
- Sudden, unexpected infant death (infant <1 yr old) (SUID) who was typically well before being placed down to sleep
- SUID includes sudden infant death syndrome (SIDS) as well as accidental suffocation and strangulation in bed (ASSB) and unknown cause of death in infants <1 yr of age
- SIDS deaths remain 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
- SIDS is distinct from ASSB which occurs when something limits a baby's breathing such as a blanket against the face or the baby is trapped between two objects (e.g., mattress and wall) which has increased since 1995.
- If nonfatal, known as BRUE (brief resolved unexplained event). Previously called ALTE (apparent life-threatening event)
- 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” program in 1994:
- 1992: 120 deaths/100,000 live births (US)
- 2001: 56 deaths/100,000 live births (US)
- No change from 2001-2008
- 2009: 54 deaths/100,000 live births (US)
- 2013: 40 deaths/100,000 live births (US)
- 2015: 39 deaths/100,000 live births (US)
- Peak occurrence of SIDS is 1-4 mo of age:
- 90% occur <6 mo of age
- 2% occur >10 mo of age
- Ethnic differences: Using 2010-2013 data, SIDS rates for non-Hispanic black and American Indian/Alaska Native populations were much higher than non-Hispanic whites, while SID rates for Asian/Pacific Island er and Hispanic populations were much lower than non-Hispanic whites
- Sleeping on back (supine) reduces incidence significantly (“Back to Sleep”). Practice of infants sleeping on their backs began initially in Europe and then in the U.S.
Etiology
- SIDS deaths are 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, and 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, breastfeeding, room sharing without bed sharing (especially in the first 6 mo), and pacifier use are protective
- Home monitoring has not been shown to prevent SIDS
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
- BRUE is associated with an increased risk of SIDS:
- An acute event that is frightening to the caretaker
- Characterized by ≥1 of the following: Cyanosis or pallor; absent, decreased, or irregular breathing; marked change in tone and altered level of consciousness
- Infant should be transported to hospital for evaluation and monitoring
- Appears well when evaluated by clinicians after recovery from BRUE
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 (e.g., BRUE), may appear well when evaluated after the episode
- Potential complications for surviving infants include pulmonary edema, aspiration pneumonia, and neurologic sequelae secondary to hypoxia, such as 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
Diagnostic Tests & Interpretation
Lab
- Selective studies based on 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
- CXR 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:
- 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
- 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
Prehospital
- 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 prehospital 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 infant's death; local, state, and national SIDS resources should be made available
- Support may be obtained from Sudden Infant Death Syndrome Alliance/First Cand le, 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
Disposition
Admission Criteria
- Observe all infants who have BRUE 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, or >1 BRUE in 24 hr
Discharge Criteria
Patients may be discharged after a BRUE if they are low risk (e.g., age >60 d, gestational age ≥7 wk and postconceptional age ≥45 wk, first BRUE, duration of event <1 min, no CPR required by trained medical provider, no historical risk factors such as changing story worrisome for child abuse, no evidence of reflux, and no concerning findings on PE)
Issues for Referral
- All surviving infants should have a pediatric consultation for support as well as to monitor for reflux
- Families will need support
- BassJL, GartleyT, LyczkowskiDA, et al. Trends in the incidence of sudden unexpected infant death in the newborn: 1995-2014 . J Pediatr. 2018;196:104-108.
- KajiAH, ClaudiusI, SantillanesG, 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.
- MitchellEA, KrousHF. Sudden unexpected death in infancy: A historical perspective . J Paediatr Child Health. 2015;51(1):108-112.
- MoonRY; Task Force on Sudden Infant Death Syndrome. SIDS and other sleep-related infant deaths: Evidence base for 2016 updated recommendations for a safe infant sleeping environment . Pediatrics. 2016;138(5):e20162940.
- OstfeldBM, Schwartz-SoicherO, ReichmanNE, et al. Prematurity and sudden unexpected infant deaths in the United States . Pediatrics. 2017;140(1):e20163334.
- TiederJS, BonkowskyJL, EtzelRA, et al. Clinical guideline: Brief resolved unexplained events (Formerly apparent life-threatening events) and evaluation of lower-risk infants . Pediatrics. 2016;137(5):e20160590.
See Also (Topic, Algorithm, Electronic Media Element)
ICD9
798.0 Sudden infant death syndrome
ICD10
R99 Ill-defined and unknown cause of mortality
SNOMED