Approach

SIDS is a diagnosis of exclusion, and is confirmed by careful postmortem evaluation. Several authors and groups have advocated for a standardized approach to the postmortem evaluation, which should include, at minimum, the clinical and pathologic components set out below.[5][116][117]

Clinical evaluation

Caregiver interview is carried out to determine the health of the child at the time of death, specifically looking for the presence of acute or chronic comorbidities that may have caused or contributed to the death (i.e., severe infections, including respiratory syncytial virus and pertussis, metabolic disease, and dysrhythmias). Symptoms could include fever, cough, nasal congestion, irritability, easy fatigability, and lethargy.

Accidental death may be established by way of evidence of overlay or suffocation in association with soft bedding or a soft sleep surface (such as a waterbed or couch/sofa/armchair), strangulation, or entrapment between 2 hard surfaces (e.g., mattress and wall).[118]

Evidence of nonaccidental or abusive traumatic injury and other forms of abuse (including Munchausen syndrome by proxy) may also be established. It is helpful in this assessment to ascertain the number of observers of incident onset and/or the level of agreement between histories provided by multiple observers, if present.

Additionally, the interview can be a time of evaluation for other identifiable SIDS risk factors, (e.g., prone, side, or inclined position at sleep; sleep position of infant when found; sleep environment; bed-sharing; prematurity; parental smoking history including maternal smoking during and after pregnancy; and history of maternal substance abuse). No factor appears to be sufficient, in itself, to initiate a SIDS event. Risk factors should be placed in the Triple-Risk hypothesis model and the aggregate of coexisting factors used to evaluate the risk of a SIDS event in any given patient. A survey of 244 SIDS cases found that 78% of cases had 2 or more concurrent risk factors (modifiable or nonmodifiable), while almost 35% had 4 or more.[61]

Family history of epilepsy, dysrhythmia, metabolic disease, and other unexplained deaths is assessed.

Care during questioning will limit the likelihood that positive responses increase caregivers' feelings of guilt and/or self-accusation. Parents and caregivers are reminded that the death was not their fault.

Laboratory evaluation/autopsy

All deaths unexplained after initial interview require a thorough and standardized autopsy.

Autopsy includes:

  • Complete microbiologic evaluation for bacterial, viral, and fungal pathogens, including analysis of blood, urine, and cerebrospinal fluid

  • Evaluation for metabolic or genetic disorders that may mimic SIDS (e.g., fatty acid metabolism disorders)

  • Photographic records of all skin and ophthalmologic findings, especially bruises and retinal hemorrhages

  • Skeletal survey to evaluate bony injuries

  • Gross internal organ and microscopic pathologic examination using a standardized autopsy protocol.

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