8 August 2013 – 4 pm
In the context of exposure to the suspected contaminated formula consider infant botulism if there is:
 new onset constipation with progression to weakness
 new onset of swallowing or feeding difficulties

 weakness, in particular affecting bulbar face and neck (typically greater than limbs) pupillary sluggishness and weak eye movements are frequent and specific findings.
For a formula-fed baby with possible constipation but no other neurological
symptoms suggestive of botulism:


Take a thorough history including formula brands used, duration of constipation, and consistency of bowel movements.
Do a thorough examination including neurological signs, abdominal palpation and visual external inspection of the rectal opening (looking for fissures or haemorrhoids/signs of straining).
 If the infant has no other symptoms apart from constipation then it is reasonable to treat the constipation with simple measures outlined below and/or laxatives (eg, lactulose, which is suitable for all ages).
 In the setting of exposure to potentially contaminated milk products but without specific symptoms or signs suggestive of infant botulism, it is advisable to follow up daily with the family, either by telephone review or in person, to ensure symptoms are resolving as expected.
Botulism is caused by the Botulinum neurotoxin (BoNT) produced by the bacteria Clostridium
botulinum. There are seven known types of BoNT (A to G) but only subtypes A, B, E and rarely F are known to cause disease in humans.
The neurotoxin causes illness by irreversibly binding to presynaptic cholinergic receptors at motor nerve terminals. This leads to a disruption in the release of acetylcholine from the presynaptic nerve that is needed to excite the muscle.
Clostridium botulinum bacteria are found throughout the environment in soil, dust and some marine environments. Clostridium botulinum spores are occasionally found in honey.
Infant botulism management 1


This more commonly affects under-six-month-olds but can affect babies up to the age of one year.
In this situation the intestinal flora is not fully developed and the spores ingested mature and start to release BoNT, which is absorbed by the intestine. Infant botulism may be difficult to recognise because of its insidious onset.
Diagnosis is largely clinical. Laboratory testing is only required in cases where infant botulism is a likely cause, and should be decided by a paediatrician.
Symptoms appear 3–30 days following ingestion. The sequence of symptoms and signs may vary but prolonged constipation (usually more than three days, and can precede weakness by several weeks) is usually the first indication. Subsequent symptoms and clinical signs include:
 poor feeding/weak suck

 lethargy and floppiness weak cry
 facial weakness

 impaired gag reflex majority are afebrile.
These may progress to:
 poor head control
 trouble swallowing saliva with excessive drooling

 generalised muscle weakness loss of ocular motility, ptosis and sluggish pupils.
Difficulty breathing and respiratory failure are late signs and can quickly lead to respiratory arrest.
The condition progresses for 1–2 weeks, and then stabilises for 2–3 weeks before recovery starts.
Typically these children do not have fever.
The symptoms are those of acute flaccid paralysis and have a differential diagnosis of infantile poliomyelitis, sepsis, meningitis, encephalitis, Guillain-Barre syndrome, some metabolic disorders, poisoning from a range of toxins, and some other neuromuscular disorders.
In the context of exposure to the suspected contaminated formula consider infant botulism if there is:
 new onset constipation with progression to weakness

 new onset of swallowing or feeding difficulties weakness in particular affecting bulbar face and neck (typically greater than limbs) pupillary sluggishness and weak eye movements are frequent and specific findings.
Infant botulism management 2

Constipation is the passage of hard dry bowel movements, not necessarily the absence of bowel movements for a few days. It is not necessarily related to the effort of passing stools. If the infant’s bowel motions are soft they are not constipated.
The normal frequency of bowel movements changes throughout infancy – it is normal for a breastfed baby to open their bowels frequently (eg, at every feed) or up to once every 7–10 days.
Formula-fed babies open their bowels between three times a day and up to once every second day.
Infants generally develop their own unique pattern of bowel movements. Deviation from this is more important than comparison to textbook figures.
Constipation is a common symptom in infancy. It is commonly associated with changing diet/milk/foods. Botulism is an extremely rare cause of constipation.
If a caregiver presents with a formula-fed baby who has possible constipation but no other
neurological symptoms suggestive of botulism:


Take a thorough history including formula brands used, duration of constipation, and consistency of bowel movements.
Do a thorough examination including neurological signs, abdominal palpation and visual external inspection of the rectal opening (looking for fissures or haemorrhoids/signs of straining).
If the infant has no other symptoms apart from constipation then it is reasonable to treat the constipation with simple measures outlined below and/or laxatives (eg, lactulose, which is suitable for all ages).
Warm relaxing baths, baby massage and laying the baby on their back and doing gentle cycling actions with their legs may be effective.
 Check that the formula is being made up as per manufacturer’s instructions. Over-concentrated formula can cause constipation. This will involve checking the volume markings on bottles are accurate by taking them to a pharmacist to be measured. It is very common for these marking to be inaccurate.
 Try a different formula brand. Some formulas are easier to digest than others. Some brands contain more iron which is constipating; changing to a brand with lower iron content may be effective.
 Return to a stage 1 formula if progressed onto a higher stage. Stage 1 formula is appropriate for all ages.
If the child is over six months old simple dietary measures are appropriate:


Introduce more plain water.
Try adding more fruit and high fibre vegetables to the diet; kiwi fruit, pear, plum, peach and apricots are particularly effective.
 Increasing fibre content without increasing water intake can make constipation worse.
If the infant is otherwise well and there are no neurological signs it is reasonable to send an infant home in these circumstances. Information should be given to caregivers regarding neurological symptoms to look out for.
Infant botulism management 3

A plan for daily follow-up either in person or over the telephone should be made for at least a week or until constipation has resolved.
For more detailed information regarding constipation in infancy and childhood see: http://www.aafp.org/afp/2006/0201/p469.html
Take a full history including suspicious food, specific formula details and other people potentially infected. Consider alternate diagnosis for acute flaccid paralysis.
Other causes need to be excluded including examination for sepsis, meningoencephalitis, electrolyte abnormalities, spinal lesions and Guillian-Barre syndrome.
Every case should be discussed with your local paediatrician. The infant is likely to need hospital admission.
If the differential diagnosis suggests bacterial sepsis then be aware that only aminoglycosides can worsen the effects of the botulism neurotoxin; other antibiotic treatment should not be delayed if indicated.
If there is evidence of respiratory distress, emergency management and respiratory support should not be delayed.
At this stage if a faecal sample is available it should be sent with the child to hospital. The paediatric team will decide on appropriate testing.
 http://www.foodsafety.govt.nz/elibrary/industry/Clostridium_Botulinum-
Neurotoxins_Produced.pdf
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 http://www.health.alberta.ca/documents/Guidelines-Botulism-2013.pdf http://emedicine.medscape.com/article/961833-overview
 http://www.cdc.gov/nczved/divisions/dfbmd/diseases/botulism
HP5699
Infant botulism management 4