SIDS, CCHS, REYE'S, HEAD INJURY PATIENT

• Sudden Infant Death Syndrome (SIDS) or Crib Death


  Usually occurs between 1 month - 1 year old
• SIDS


  • Common in preterm - SGA, male non-whites like black, native American or Alaskan
  • Happens while asleep especially during winter months
  • Usually second or third in birth order or of a multiple birth
  • Previous siblings SIDS victim
• Probable etiologies of SIDS

  • Born to a poor mother less than 20 y.o. who received inadequate prenatal care with history of smoking or drug addiction (narcotics)
  • Recent bout of upper respiratory tract infection or URTI (50%)
  • Airway obstruction during sleep
  • Triggering of mammalian diving reflex (results to apnea and bradycardia)
  • Prone sleeping position
  • History of apparent life-threatening events (ALTE) - apnea, poor skin/muscle tone, gagging/choking

  
• Interventions/Preventions for SIDS or Crib Death

  1. Identify high risk infant based on history

  2. Apply CPR as needed

  3. Put patient on pneumograms or apnea monitors

  4. Infants be placed on supine or side-lying positions on the first (6) months of extrauterine life
• Congenital Central Hypoventilation Syndrome (CCHS)


  Life-threatening disorder manifesting as sleep-associated alveolar hypoventilation or apnea
• CCHS


  • Should be considered in children with episodic or sustained hypoventilation and hypoxemia in the first months of life when asleep without obvious metabolic, cardiopulmonary, or neuromuscular disease
  • Can have an early onset (first months) or late onset (school age or adulthood) onset
• CCHS


  • Genetically linked (autosomal dominant) condition
  • A mutated gene known as PHOX2B from a parent can be inherited by a child
  • A very rare life-long disorder; about 1:200,000

  
• CCHS is also associated with

  • Generalized dysfunction of the autonomic nervous system, including cardiovascular and ophthalmic regulation
  • Hirschsprung disease - absent/impaired bowel function; associated with 20% of CCHS cases (Hadad syndrome)
  • Neural crest tumors - associated with 5-10% of cases
• Clinical Manifestations in CCHS

  • Apnea or hypoventilation during sleep with no other underlying conditions
  • Hypotonia - younger patients
  • Cyanosis
  • Development of cor pulmonale, edema, and pulmonary hypertension
  • Fluctuations in BP (wakefulness - lower; asleep - goes up); sinus bradycardia
  • Diaphoresis; hypothermia
  • Mild cognitive defects - older patients
  • Ocular findings (miosis, anisocoria, abnormally responsive to light)
  • Gastroesophageal reflux, decreased intestinal motility and constipation
• Treatment and Management for CCHS

  1. Lifelong (annual/biannual) evaluation-monitoring of the child with appropriate medication or procedure if needed: Ventilatory status, Heart function, ANS and Neurocognitive assessment, Glucose monitoring, Gastrointestinal function

  2. Mechanical ventilation: Invasive - during sleeping hours; patient is tracheostomized, Non-invasive - for older patients >7 years

  3. Diaphragm pacing - rhythmic electrical stimulation
• Reye's Syndrome


  A potentially fatal disease that has numerous detrimental effects to many organs, especially the brain and liver

  
• Reye's Syndrome


  • Classic features: rash, vomiting, behavioral changes, sleepiness and liver damage
  • Exact cause is unknown; it has been associated with aspirin (salicylic acid) consumption by children with viral illness, but it also occurs in the absence of aspirin use

  
• Reye's Syndrome


  • Fatty liver with minimal inflammation (enlargement) and firmness
  • Jaundice is rare
  • Severe encephalopathy (swelling of the brain)
  • Edema and fatty degeneration of proximal tubules in the kidneys
• Early diagnosis is vital; although most children recover with supportive therapy, it may lead to severe brain injury and death
• Treatment of Reye's Syndrome

  1. IVF for Glucose/ electrolyte imbalances

  2. Treatment of brain swelling aimed at lowering the serum ammonia level

  3. Vitamin K, plasma platelet concentrate for treatment of bleeding

  4. Hemodialysis – to remove toxins believed to be partly responsible for the brain swelling

  5. Cardiopulmonary status monitoring and nutrition intake

  6. Intracranial Pressure (ICP) and electrolyte monitors

  7. Insulin, corticosteroids; diuretics for ICP and medication for seizures

  8. Mechanical ventilation may be indicated
• Diagnostics - CT scan/MRI of the brain, liver profile testing
• Most pediatric head trauma occurs secondary to

  • motor vehicle accidents
  • falls
  • assaults
  • recreational activities
  • child abuse
• Patients with severe head trauma are at increased risk of developing

  • cerebral edema
  • respiratory failure
  • brain herniation secondary to increased ICP
• Primary survey


  • Focused on physical examination of:
  • Airway (i.e. presence of foreign bodies, facial lacerations, bone instability, tracheal deviation, circumoral cyanosis)
  • Breathing (i.e. apnea, hypoventilation)
  • Circulatory status (i.e. Cushing triad of bradycardia, hypertension, altered respiration)
  • Neurologic status (i.e. alert, verbal, pain, unresponsive [AVPU] system; pediatric Glasgow Coma Scale [GCS])
• Glasgow Coma Scale


  Assesses neurologic status

  
• Secondary survey


  • Detailed assessment of body systems including:
  • HEAD/NECK (i.e. cervical deformity, step-off or malalignment, lacerations, depressions, battle sign or retroauricular/mastoid ecchymosis, raccoon eyes/periorbital ecchymosis, hemotympanum, cerebrospinal fluid otorrhea and rhinorrhea, bulging of fontanel)
  • Respiratory patterns (i.e. apnea, Cheyne-Stokes, hyperventilation, apneustic respiration)
  • Neurologic status (i.e. Dilated (mydriasis)/pinpoint (miosis) pupil(s), Horner syndrome – ptosis (drooping of eyelids), miosis, anhidrosis, eye/retinal hemorrhage, motor/sensory dysfunction)
• Care of Neurologically Impaired Child - Head Injury Management

  1. Airway care; intubation – possible mechanical ventilation

  2. Cardiovascular/circulatory status monitoring

  3. Control of ICP and cerebral perfusion

  4. Control of bleeding

  5. Anti-seizure drugs – phenobarbital (Luminal), phenytoin (Dilantin)

  6. Temperature control

  7. Analgesia, sedation, and neuromuscular blockade