SUDDEN INFANT DEATH SYNDROME

Sudden infant death syndrome is defined as quot;the unexpected death of an infant (lt;1 year), the cause of which remains unexplained after an autopsy, death scene investigation and review of case history”.

While incidence of SIDS has declined in recent years, it continues to be an important cause of infant mortality

TABLE 27.29: Risk factors for SIDS

TABLE 27.30: Causes of ALTS

• Family history of SIDS

• History of acute life threatening events*

• Infant-related

- Age (90% cases in 1-6 months group)

- Prematurity

- Male gender

- Recent febrile illness

- Tendency to sleep in prone position

• Maternal factors:

- Young, undernourished or ill mothers

- Unwed mothers

- Repeated pregnancy at short intervals

- Smoking or substance abuse

• Environmental factors:

- Low socioeconomic status

- Winter season

- Use of soft-bedding

*discussed later

in developed countries with an estimated incidence of ~ 0.7/1000 live births.

Risk factors: Most of these deaths occur during first 6 months of life in previously healthy infants, usually during sleep at night and in winter season (Table 27.29). Pathophysiology of SIDS is still obscure, though seems to revolve around the maturational delay or pathological abnormalities in cardiorespiratory regulation during sleep, due to:

• Impaired brain-stem arousal response to sleep-related asphyxia,

• Impaired chemoreceptor sensitivity to hypercarbia or hypoxia,

• Impaired respiratory response, e.g. prolonged periodic breathing

• Impaired heart-rate variations or arrhythmias during sleep, ? due to decreased vagal tone,

• Autonomic dysfunction in response to temperature changes during sleep.

Management: As SIDS is a fatal event, management involves: (a) parental counseling, specially to remove their guilt, (b) detailed review of previous health records and autopsy findings to exclude identifiable causes of death or epidemiological risk factors, (c) prospective care during next pregnancy with elimination of high- risk factors.

A simple intervention, e.g. campaign to avoid prone position during sleep has reduced incidence of SIDS in United States by ~ 50%.

Acute life-threatening events (ALTS) or near-missed SIDS, are defined as “an un-expected and frightening change in infants' behavior, characterized by apnea, color change, limpness and chokingquot;. These events are more common than SIDS (0.5-1/1000 live births) and cause is identifiable in ~50% of these cases after detailed investigations (Table 27.30). However, an infant with

• Idiopathic (50%)

• Cardiac: CHDs, long-QTc syndrome, arrhythmia

• Resp.: GER, airway anomalies, chronic lung disease

• CNS: Seizure disorder, brain-stem lesions

• Metabolic: Hypoglycemia, IEM*

• Infections: Acute fulminant sepsis, meningitis

• Trauma to vital organs e.g. head injury

*GER: Gastro-esophageal reflux; IEM: Inborn errors of metabolism; e.g. carnitine deficiency, urea cycle defects and fatty acid disorders

ALTS carries 3-5 times higher risk of subsequent SIDS and needs to be investigated and treated for identifiable cause with follow-up in early childhood.

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