Pediatric Pearls – Floppy Baby

Pediatric Pearls – Floppy Baby

Ilene Claudius MD and Sol Behar MD 

Take Home Points

• Botulism is a clinical diagnosis and early initiation of appropriate treatment may result in better outcomes.
• Fatigable pupils are associated with botulism.
• Construction, earthquakes and honey containing products or home remedies have been associated with botulism.
• Constipation is often the initial presenting symptom of infantile botulism.

Case

• Behar was working with one of his chief residents. They saw a seven month old baby brought in by the mother. The mother reported that although the baby had been able to sit up by himself previously, he was no longer able to do so. She opened her purse and it was filled with homeopathic remedies. She wasn’t very concerned. She reported initially the baby would fall to the side, but now he just didn’t have the tone to keep himself up.
• The baby did not have any fever, cough or recent illness. No vomiting or diarrhea. He hadn’t pooped for a few days which was normal for him (and one of the reasons she put him on probiotics). He choked when he was drinking milk.
• The baby was in the mom’s arms. He was like a rag doll sliding through her arms. By 7 months, he should have enough tone to cling to the mother like a koala bear. When he laid down, he was like a starfish with his arms and legs extended. When lifted by his arms, his head lagged behind. He was globally hypotonic.
• Alternative or complementary medications aren’t tested or regulated in the way actual medications are. They don’t have to be shown to work or to be safe. They don’t even have to contain the ingredients listed on the label. Claudius does not recommend the use of these products in children this age.
• The child was afebrile. His vital signs were normal. His heart and lungs were unremarkable. He looked like Garfield the cat. He had a profound ptosis. Both eyelids were at half-mast. He was globally floppy and vocalizing. He was attentive but looked sleepy and hypotonic.
• What is your differential diagnosis?
  • Acute flaccid paralysis most commonly affects one or two limbs.
  • Congenital conditions such as spinal muscular atrophy usually present earlier although there are different types. This is characterized by absence of reflexes.
  • Claudius has seen some weird cases of myasthenia gravis in children.
  • The child was given supplements and you need to consider botulism.
• What did they do? Behar called the neurologist. If you do think it is infantile botulism, it is a clinical diagnosis and the earlier you give the baby the immunoglobulin, the better the outcome may be. When Behar shined the light in the child’s eyes, they constricted normally. When he held the light there, the pupils began to dilate. Fatigable pupils are specific for botulism.
• What is the botulism toxin and how does it work? The toxin causes a functional denervation of skeletal and smooth muscle. It happens at the synaptic level. It binds to the presynaptic membrane and prevents the release of acetylcholine. You can’t get neurotransmission and it results in global weakness. This manifests in smooth muscle as constipation. This is almost always the initial presenting sign of infantile botulism. This may be followed by descending weakness, global hypotonia and cranial nerve abnormalities such as ptosis. They may develop automatic instability, diaphragmatic paralysis and respiratory failure.
• Why does the treatment differ in infants? Babies don’t have the stomach acid or motility to prevent the spore from forming toxin. If you can get the immunoglobulin on board, you can prevent complications and decrease the duration of illness.
• What happens if you don’t give BabyBIG (botulism immunoglobulin)? This is expensive at about $45,000 per dose. You may need neurology approval to order it. If you let this go, the typical duration of disease is about 2 months. Occasionally, there may be relapses. These children will likely require ICU and tube feeds. If treatment is initiated early, you may have a good outcome.
• What are causes infantile botulism? Being near a construction site or earthquake where spores are aerosolized. Giving honey. Some of the cough and cold remedies have honey in it. Honey is only responsible for about 15% of cases but is a more easily modified risk factor.
  • There is a new pacifier on the market that contains honey.
• Additional history was obtained when the patient was in the ICU. The family had been redoing their driveway. The illness was more likely due to spores kicked up from the construction than homeopathic remedies.