II. Epidemiology
- See Botulism
-
Incidence: Estimated at 70 cases of Infant Botulism in U.S. per year
- More cases than foodborne or Wound Botulism
- States with highest rates
- California (50%)
- Utah
- Pennsylvania
- Age of Onset
- Age 6 weeks to 9 months
- Peaks at 2-3 months (90% are under 6 months of age)
III. Pathophysiology
- See Botulism
-
Botulinum Toxin binds at presynaptic membrane and prevents Acetylcholine release
- Results in a functional denervation of skeletal and Smooth Muscle
- Sources
- Contaminated soil (e.g. construction site, farm, earthquake)
- Contaminated honey (10% of samples)
- Contaminated corn syrup (0.5% of samples)
- Vacuum cleaner dust
- Other related factors
- Infant gastric acid and gastric motility fails to prevent botulinum spore germination and toxin release
- Infants under 2 months living in rural farming area
- Infants over 2 months are typically Breast fed
- Nursing infants account for 70-90% Infant Botulism
- Nursing may be protective and delay severity
- Non-nursing infants may have fatal undiagnosed case
- Intestinal Botulism (primary source of toxin in infants)
- Botulinum spores germinate and colonize the infant intestinal tract
- May result in a prolonged Incubation Period (e.g. weeks)
- Botulinum Toxin is produced in the infant colon
- Intestinal Botulism is unique to infants
- Older children and adults have intestinal tracts colonized by competitive Bacteria
- Competitive organisms prevent botulinum colonization in older children and adults
- May occur in adults with altered GI Tract (e.g. Gastric Bypass surgery, Proton Pump Inhibitors)
- Botulinum spores germinate and colonize the infant intestinal tract
IV. Findings: Symptoms and Signs
- See Botulism
- Early symptoms and Signs
- Constipation (65%)
- May precede weakness by days
- Cranial Nerve Dysfunction (subtle at onset)
- Weak cry and weak suck
- Loss of facial expression
- Decreased oral intake (79%)
- Decreased Gag Reflex
- Cranial Nerve 6 Palsy (unable to abduct eye)
- Mydriasis with sluggish pupil reaction
- Ptosis
- Autonomic changes
- Hypotension
- Neurogenic Bladder
- Constipation (65%)
- Later Symptoms and Signs (typically by day 4 of illness)
- Descending weakness, flaccidity, Floppy Infant or hypotonia (88%)
- Poor head control
- Decreased activity or lethargy (60%)
- Irritability
- Respiratory difficulties
V. Differential Diagnosis
- See Hypotonia in Infants (Floppy Infant)
- See Pediatric Constipation Causes
- Sepsis
- Guillain Barre
VI. Labs
- See Botulism
- Serum sample for Botulinum Toxin
-
Stool for toxin and culture
- Passed stool is preferred
- Sample (25 g or 25 ml) via colonic irrigation
- Possible sources sent for Botulinum Toxin
- Dust or soil from clothing
- Honey, Corn syrup and other foods
VII. Diagnosis
- See Botulism
- Electromyogram (EMG)
VIII. Management
- Supportive care with close supervision
- Monitor respiratory function closely
- Anticipate Mechanical Ventilation
-
Antibiotics are not recommended
- Penicillin G (or Metronidazole) is used only for Wound Botulism (older children and adults)
- Consider Botulinum Immune globulin (Baby BIG)
- Efficacy
- Reduces hospitalization duration
- Reduces Mechanical Ventilation duration
- Source: California Department of Public Health (CDPH Infant Botulism Program)
- Efficacy
- Botulinum antitoxin
- Not recommended in infants
- Botulinum Immune Globulin is preferred over antitoxin
- May not be beneficial in Infant Botulism
- Anaphylaxis rate with trivalent Vaccine was very high (9 to 20%)
IX. Prognosis
- Infant mortality in unrecognized cases is typically due to Acute Respiratory Failure
- Among the causes of Sudden Infant Death Syndrome (SIDS)
- Case fatality rate of treated patients: <2%
- Excellent long-term prognosis without residual changes
X. Course
- Mechanical Ventilation: 23 days
- Hospital stay on average: 44 days
- Relapses, if they occur, usually do so within 13 days
XI. References
- (2019) Sanford Guide, acccessed 6/5/2019
- (2000) AAP Red Book, 25th edition, p. 212-13
- Della-Giustina (2024) Crit Dec Emerg Med 38(10): 27-34
- Schechter in Behrman (2000) Nelson Pediatrics, p. 875-8
- Cox (2002) Am Fam Physician 65(7):1388-92 [PubMed]
- Muensterer (2000) Pediatr Rev 21(12):427 [PubMed]