A 7-day-old term female infant presented to the emergency room with several hours of decreased energy and poor feeding.
She is noted to be grey in color with poor perfusion, decreased tone, weak cry, and no respiratory distress but poor effort.
Resuscitation was begun. She had intravenous catheters placed with fluid boluses given and ampicillin, gentimicin and acyclovir begun.
The pertinent physical exam showed a heart rate of 100, blood pressure of 52/28, respiratory rate of 40, and a pulse oximetery was not registering.
Her pupils were 7-8 mm and not reactive to light. Her fontanelle was normal. There was no rash, or bruises. Capillary refill was 3 seconds.
Cardiac examination revealed normal S1, S2 and no murmur. Abdomen was soft with no obvious organomegaly.
Neurological examination revealed decreased tone, withdrawal to pain, and her peripheral reflexes were difficult to elicit.
The laboratory evaluation showed a first blood gas with a pH = 7.14, CO2 = 56, O2 = 80. Blood cultures were drawn. She had a normal complete blood count, andelectrolytes including calcium, magnesium and phosphorus.
The patient was then intubated and transferred to a regional children’s hospital.
Additional work-up included a normal cerebral spinal fluid, and a normal computerized tomography of the head and chest radiograph.
An electrocardiogram and echocardiogram were also normal.
She had a normal electroencephalogram but an abnormal electromyelogram which was consistent with a peripheral versus a polymyopathic neuropathy. She did not respond to a tensilon test.
The additional past medical history revealed normal labor and delivery at home for a G1P1 mother with some prenatal care.
The patient was seen by a nurse midwife at delivery, day 1 and day 2 of life and was doing well. Her neonatal screen was normal. She did not receive Hepatitis B, Vitamin K or gonococcal conjunctivitis prophylaxis.
She was breastfeeding well until several hours before coming to the emergency room.
The parents denied honey ingestion and the family history revealed no neuropathy or neurological diseases.
The diagnosis of presumed infantile botulism was made and the infant was given botulinum antitoxin.
At day 14, Clostridium botulinum type A was recovered from her stool and she remained on life support. She was discharged home after a few weeks.
A newborn infant presenting with acute deterioration causes health care providers to quickly go through a long differential diagnosis covering all organ systems as they attempt to resuscitate and stabilize the infant.
Causes are numerous and the physicians in this case entertained some of the following in their differential:
- Sepsis/Infection – Group B streptococcal infection, Herpes simplex, necrotizing enterocolitis, etc.
- Congenital heart disease
- Hematologic – bleeding secondary to sepsis, Vitamin K deficiency, etc.
- Metabolic abnormalities – endocrinopathies, nephropathies, inborn error of metabolism, etc.
- Neurologic – seizures, congenital or acquired neuropathies, etc.
- Trauma – inflicted or incidental
Like most patients, the cause for this patient’s acute deterioration not immediately recognized.
This patient was resuscitated, stabilized, transported, and then was sustained with ventilatory support and other life sustaining measures for the most common problems such as sepsis and congenital abnormalities.
Concurrently, her evaluation was begun and based on those results the differential was narrowed down over time and specific treatment initiated.
Infantile botulism is not a common problem in the United States generally because of improved obstetrical care, general personal hygiene and education about cultural practices which could be harmful to the infant such as giving honey or placing soil or dung on the umbilical cord.
Botulism is classified into four categories – foodborne, infantile, wound and undetermined. Symptoms (except for infants) generally occur within hours and evolve over days. It is a decending, symmetric, flaccid paralysis, with cranial nerves palsies being the most common complication.
Infantile botulism occurs most often in children < 6 months and can be preceeded by constipation, poor feeding, weak cry, ocular nerve palsies, general weakness and hypotonia.
Human botulism is associated with neurotoxins A, B, E and F. Infantile botulism is almost always associated with types A and B.
Incubation in infantile botulism is 3-30 days from exposure.
About 100 cases occur annually of infantile botulism, after ingested spores of Clostridium botulinum are ingested, germinate, replicate and produce toxin in the gut. The toxin produces the paralysis.
In infantile botulism the source of the spores sometimes cannot be identified and possibly is airborne dust or soil. Honey often is not certified as free of spores and should be avoided especially in the first year of life.
Corn syrups are manufactured under sterile conditions, but are not packaged under aseptic conditions and do not receive sterilization at the end of packaging and therefore the manufacturers cannot ensure that the product is free of spotes.
Antibiotics are avoided if possible because Clostridium lysis may increase the amount of toxin. Botulinum antitoxin (human-derived immunoglobulin) is available and in studies decreases the hospitalization length. Overall mortality is ~15%.
Questions for Further Discussion
1. Clostridium botulinum is a Class A bioterrorism agent. What are the other Class A agents?
2. How is Botox® (Botulinum toxin Type A) made safe for human use?
3. How are the presentations of foodborne and wound botulism different from the infantile form?
To Learn More
To view pediatric review articles on this topic from the past year check PubMed.
Information prescriptions for patients can be found at MEDLINEplus for this topic: Botulism
To view current news articles on this topic check Google News.
American Academy of Pediatrics. Botulism and Infant Botulism, In Pickering LD, ed. Red Book: 2003 Report of the Committee on Infectious Diseases. 26th edit. Elk Grove Village, IL: American Academy of Pediatrics; 2003;243-245.
FrattarrelliDAC, Abdel- Haq NM. Botulism. eMedicine.
Available from the Internet at http://www.emedicine.com/ped/topic273.htm (rev. 7/9/2004, cited 6/1/06).
ACGME Competencies Highlighted by Case
1. When interacting with patients and their families, the health care professional communicates effecively and demonstrates caring and respectful behaviors.
2. Essential and accurate information about the patients is gathered.
3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
4. Patient management plans are developed and carried out.
7. All medical and invasive procedures considered essential for the area of practice are competency performed.
9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.
10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.
13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.
22. Sensitivity and responsiveness to patients’ culture, age, gender, and disabilities are demonstrated.
26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.
Donna M. D’Alessandro, MD
Associate Professor of Pediatrics, Children’s Hospital of Iowa
June 19, 2006