When Timing is Everything: A Case Report of Prompt Treatment and Recovery for Infant Botulism

by Emma Livne, MD (’24)

Case 

A 7-week-old female infant who was born full term with no PMH and normal newborn screen presented to the ED with 3 days of decreased oral intake, constipation, poor suck, weak cry, and hypotonia. She presented while on vacation, shortly after arriving to Rhode Island from their home in New Jersey. Significant head lag, decreased energy, weak cry and poor suck were noted on exam, with normal vital signs for age. A sepsis work-up was obtained and she was admitted to the pediatric hospital medicine service on broad spectrum antimicrobials. CBC, CMP, CRP, UA and CSF studies were normal. Blood, urine, and CSF cultures were negative. Head CT was normal. She was rhino/enterovirus positive, however, CSF enterovirus PCR was negative. Her exam progressed to diffuse symmetric hypotonia with bilateral ptosis, dilated and sluggishly reactive pupils, decreased gag reflex, poor suck, and diminished reflexes.

 Diagnosis and Initial Management

Due to concern for infantile botulism, baby botulism immunoglobulin (BabyBIG) was requested from the Infant Botulism Treatment and Prevention Program, which required shipment from out-of-state, and antibiotics were discontinued. That evening she was transferred to the PICU for respiratory monitoring due to intermittent bradypnea though she did not require respiratory support. An NG tube was placed for feeds. She received BabyBIG on day 2 of hospitalization and stool samples for botulinum toxin were sent to the CDC.

 Background

        Infantile botulism is caused by ingestion of spores from the gram-positive anaerobe Clostridium botulinum, typically found in honey or soil.

       It is characterized by symmetric, descending flaccid paralysis of the motor and autonomic nerves, always beginning with cranial nerve palsies.

       Signs and symptoms in an infant may include:

o    Constipation

o    Poor feeding

o    Ptosis or sluggish pupils

o    Flattened facial expression

o    Diminished suck and gag reflexes

o    Weak and/or altered cry

       Diagnosis is made clinically and is treated empirically while awaiting confirmatory testing to avoid complications.

 

Discussion

       If left untreated, botulism has a mortality rate of 40-50%. Disease outcome has improved with the use of mechanical ventilation and the development of anti-toxin3.

       In 2003 the FDA approved the use of human botulinum immune globulin intravenous (BabyBIG) for the treatment of infant botulism.

       Infant botulism is rare in RI, with incidence of 2-4 cases in 10 years (2009-2018), relative to 78-80 in NJ2.

       Inpatient hospitalizations for cases treated with BabyBIG have a mean duration of 2.6 weeks with average duration of tube feeding of 3.6 weeks3.

       Treatment with BabyBIG significantly shortens duration of hospital stay, tube feeding, and mechanical ventilation, with increased effectiveness if given within 72 hours of hospitalization3.

       BabyBIG treatment within 3 days of hospitalization as compared to 7 days results in a mean length of hospital stay of 2.0 weeks, as compared to 2.9 weeks, respectively3.

 

Case Resolution

The infant improved unexpectedly over the next 72 hours, bringing the working diagnosis into question. A differential including EVD68 acute flaccid paralysis, underlying metabolic disorder/myopathy, or rare congenital neuromuscular disorders were considered. An MRI brain was completed and normal. EVD68 samples were collected and frozen, though ultimately not sent as the stool sample returned positive for Botulinum Type B, the predominant serotype in regions east of the Mississippi River. She was discharged home after a 9 day hospitalization, taking full feeds by mouth. Discharge exam was significant for head lag and mild hypotonia but findings were otherwise resolved.

This infant’s case is notable for her rapid improvement in symptoms and short hospitalization, illustrating the importance of a high index of suspicion and prompt treatment for infants with botulism.

 

 References

  1. Arnon, S. S., Schechter, R., Maslanka, S. E., Jewell, N. P., & Hatheway, C. L. (2006). Human botulism immune globulin for the treatment of infant botulism. New England Journal of Medicine, 354(5), 462–471. https://doi.org/10.1056/nejmoa051926
  2. Centers for Disease Control and Prevention (CDC). Botulism Annual Summary, 2018. Atlanta, Georgia: U.S. Department of Health and Human Services, CDC, 2021.
  3. Chalk, C.H., Benstead, T. J., Pound, J. D., & Keezer, M. R. (2019). Medical treatment for botulism. Cochrane Database of Systematic Reviews. https://doi.org/10.1002/14651858.cd008123.pub4

 

Acknowledgements

  •         Kelsey hart, MD; Edward Gill, MD; Molly Taylor, MD; and Mary Tarantino, PhD
  •        Hasbro Children’s Hospital and the Brown Pediatric Residency Program
  •       Special thanks to this patient and her famil