Infant Botulism

Aka: Infant Botulism, Infantile Botulism, Intestinal Botulism

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II. Epidemiology

  1. See Botulism
  2. Incidence: Estimated at 70 cases of Infant Botulism in U.S. per year
    1. More cases than foodborne or Wound Botulism
  3. States with highest rates
    1. California (50%)
    2. Utah
    3. Pennsylvania
  4. Age of Onset
    1. Age 6 weeks to 9 months
    2. Peaks at 2-3 months (90% are under 6 months of age)

III. Pathophysiology

  1. See Botulism
  2. Botulinum Toxin binds at presynaptic membrane and prevents Acetylcholine release
    1. Results in a functional denervation of skeletal and Smooth Muscle
  3. Sources
    1. Contaminated soil (e.g. construction site, farm, earthquake)
    2. Contaminated honey (10% of samples)
    3. Contaminated corn syrup (0.5% of samples)
    4. Vacuum cleaner dust
  4. Other related factors
    1. Infant gastric acid and gastric motility fails to prevent botulinum spore germination and toxin release
    2. Infants under 2 months living in rural farming area
    3. Infants over 2 months are typically Breast fed
      1. Nursing infants account for 70-90% Infant Botulism
      2. Nursing may be protective and delay severity
      3. Non-nursing infants may have fatal undiagnosed case
  5. Intestinal Botulism (primary source of toxin in infants)
    1. Botulinum spores germinate and colonize the infant intestinal tract
      1. May result in a prolonged Incubation Period (e.g. weeks)
    2. Botulinum Toxin is produced in the infant colon
    3. Intestinal Botulism is unique to infants
      1. Older children and adults have intestinal tracts colonized by competitive Bacteria
      2. Competitive organisms prevent botulinum colonization in older children and adults
      3. May occur in adults with altered GI Tract (e.g. Gastric Bypass surgery, Proton Pump Inhibitors)

IV. Findings: Symptoms and Signs

  1. See Botulism
  2. Early symptoms and Signs
    1. Constipation (65%)
      1. May precede weakness by days
    2. Cranial Nerve Dysfunction (subtle at onset)
      1. Weak cry and weak suck
      2. Loss of facial expression
      3. Decreased oral intake (79%)
      4. Decreased Gag Reflex
      5. Cranial Nerve 6 Palsy (unable to abduct eye)
      6. Mydriasis with sluggish pupil reaction
      7. Ptosis
    3. Autonomic changes
      1. Hypotension
      2. Neurogenic Bladder
  3. Later Symptoms and Signs (typically by day 4 of illness)
    1. Descending weakness, flaccidity, Floppy Infant or hypotonia (88%)
    2. Poor head control
    3. Decreased activity or lethargy (60%)
    4. Irritability
    5. Respiratory difficulties

V. Differential Diagnosis

  1. See Hypotonia in Infants (Floppy Infant)
  2. See Pediatric Constipation Causes
  3. Sepsis
  4. Guillain Barre

VI. Labs

  1. See Botulism
  2. Serum sample for Botulinum Toxin
  3. Stool for toxin and culture
    1. Passed stool is preferred
    2. Sample (25 g or 25 ml) via colonic irrigation
  4. Possible sources sent for Botulinum Toxin
    1. Dust or soil from clothing
    2. Honey, Corn syrup and other foods

VII. Diagnosis

  1. See Botulism
  2. Electromyogram (EMG)

VIII. Management

  1. Supportive care with close supervision
    1. Monitor respiratory function closely
    2. Anticipate Mechanical Ventilation
  2. Antibiotics are not recommended
    1. Penicillin G (or Metronidazole) is used only for Wound Botulism (older children and adults)
  3. Consider Botulinum Immune globulin (Baby BIG, BIG-IV)
    1. BIG-IV is a human serum derived product that does not predispose to Serum Sickness or Anaphylaxis
      1. Contrast with Adult Botulinum antitoxin which is derived from equine serum
    2. Efficacy
      1. Reduces hospitalization duration
      2. Reduces Mechanical Ventilation duration
    3. Source: California Department of Public Health (CDPH Infant Botulism Program)
      1. https://www.cdph.ca.gov/Programs/CID/DCDC/Pages/ObtainBabyBig.aspx
  4. Adult Botulinum antitoxin (equine serum)
    1. Not recommended in infants
    2. Botulinum Immune Globulin is preferred over antitoxin
    3. May not be beneficial in Infant Botulism
    4. Anaphylaxis rate with trivalent Vaccine was very high (9 to 20%)

IX. Prognosis

  1. Infant mortality in unrecognized cases is typically due to Acute Respiratory Failure
    1. Among the causes of Sudden Infant Death Syndrome (SIDS)
  2. Case fatality rate of treated patients: <2%
  3. Excellent long-term prognosis without residual changes

X. Course

  1. Mechanical Ventilation: 23 days
  2. Hospital stay on average: 44 days
  3. Relapses, if they occur, usually do so within 13 days

XI. References

  1. (2019) Sanford Guide, acccessed 6/5/2019
  2. (2000) AAP Red Book, 25th edition, p. 212-13
  3. Della-Giustina (2024) Crit Dec Emerg Med 38(10): 27-34
  4. Schechter in Behrman (2000) Nelson Pediatrics, p. 875-8
  5. Cox (2002) Am Fam Physician 65(7):1388-92 [PubMed]
  6. Muensterer (2000) Pediatr Rev 21(12):427 [PubMed]

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