II. Epidemiology

  1. Incidence: Estimated at 250 cases in U.S. per year
    1. More cases than foodborne or Wound Botulism
  2. States with highest rates
    1. California (50%)
    2. Utah
    3. Pennsylvania
  3. 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. 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

IV. Findings: Symptoms and Signs

  1. See Botulism
  2. Early symptoms and Signs
    1. Constipation (65%)
      1. May precede weakness by weeks
    2. Cranial Nerve Dysfunction
      1. Weak cry and weak sucking
      2. Decreased oral intake (79%)
      3. Decreased Gag Reflex
      4. Cranial Nerve 6 Palsy (unable to abduct eye)
      5. Mydriasis with sluggish pupil reaction
      6. Ptosis
    3. Autonomic changes
      1. Hypotension
      2. Neurogenic Bladder
  3. Later Symptoms and Signs
    1. Descending weakness, flaccidity, Floppy Infant or hypotonia (88%)
    2. Decreased activity or lethargy (60%)
    3. Irritability
    4. Respiratory difficulties

V. Differential Diagnosis

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

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)
    1. Efficacy
      1. Reduces hospitalization duration
      2. Reduces Mechanical Ventilation duration
    2. Source: California Department of Health Services
      1. Phone (24 hours): 510-540-2646
  4. Botulinum antitoxin
    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. Case fatality rate of treated patients: <2%
  2. 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. Schechter in Behrman (2000) Nelson Pediatrics, p. 875-8
  4. Cox (2002) Am Fam Physician 65(7):1388-92 [PubMed]
  5. Muensterer (2000) Pediatr Rev 21(12):427 [PubMed]

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