Interesting Case

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Homunculus

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6 month old male, presents acutely to clinic (or your ED) with a chief complaint of "not feeding as much" and "not being as active as normal" over the past 3-4 days. Mom says he "feels hot" sometimes but hasn't taken a temp. Term, SVD, uncomplicated pregnancy, vaccines all up to date. Exclusively breastfed and recently introduced veggies. No GI symptoms, last stool 2d ago. No bloody diarrhea/constipation/vomiting. 1 older sib (5yo) in home. On presentation, is lethargic but actively following observers in the room, is tachypneic, tachycardic, has poor cap refill, and is mottled. Right TM bulging/erythematous/non-mobile, left erythematous. remainder of exam in unremarkable with normal neuro exam.

blood cx, urine cx, LP, CBC, UA, Bolus NS, Rocephin, IVF started. 10 minutes after abx/bolus, desats to low 90's. HR improved, liver edge still normal, cap refill improved. 2nd bolus given. Sats slowly improve to upper 90's. Slightly more spontaneous movement, but still lethargic.

Transferred to ward. On ward desats again and fails to improve with mask. transferred to PICU and intubated. Pt noted to to have decreased gag, be more hypotonic, decreased DTR's compared to prior exam, and has dilated pupils.

questions/ideas?

--your friendly neighborhood riddler caveman
 
What race/ethnicity?
 
botulism? were those home canned veggies?

not home canned veggies. but yeah, botulism.

apparently the only place to get babyBIG (botulism immunoglobulin) is from CA. and they charge a pretty penny-- roughly $45K for a single treatment. (wire transfer only-- seriously :laugh:) it halts progression, but care is still supportive until he regenerates his motor end plates. he was toxin B positive in his stool. only approx 15% or so of infant botulism cases are classic "honey" cases-- the rest are thought to be environmental. the closest thing he had to an exposure is a new house being built 1/2 mile away, and he was caught chewing on his brother's dirty shoes about a week prior.

we nailed it early, but only because one of our attendings had seen it before present almost the exact same way. either way, intubation and time woulda healed him.

--your friendly neighborhood toxin chasing caveman
 
botulism? were those home canned veggies?

one more thing-- i shoulda guessed someone from the goljan sphere of influence would have honed in on the veggies. i shoulda just left that part out since it was irrelevant, the case woulda lasted longer 😛

--your friendly neighborhood amish boltulism infested canned mushroom eating caveman
 
🙂

I just found this on emedicine & thought it was interesting:
"Breastfeeding may protect infants from lethal fulminant infant botulism, but exclusive breastfeeding is a risk factor for the disease, presumably because the relatively pristine bowel flora of the exclusively breastfed infant is more permissive for spore germination and toxin production."
 
I love it when y'all post cases.
 
🙂

I just found this on emedicine & thought it was interesting:
"Breastfeeding may protect infants from lethal fulminant infant botulism, but exclusive breastfeeding is a risk factor for the disease, presumably because the relatively pristine bowel flora of the exclusively breastfed infant is more permissive for spore germination and toxin production."


there was an article in Pediatrics a while back comparing intestinal flora of breastfed to bottlefed infants (term and preterm) and found that breastfed kiddos had more beneficial and less potentially pathogenic bacteria

there's also a potential risk w/ aminoglycosides worsening the situation-- but we rarely use those in kids outside the NICU or nursery anyway.

--your friendy neighborhood gut bug studying caveman
 
Classic scenario for bo tox: limp baby, eyes still tracking.
 
yeah, this eye findings were definitely odd-- enough so they were documented in the original exam. tone had pretty much normalized after a couple of boluses-- decreased spontaneous movement, but still had some intentional movement toward objects, was grasping, and had normal reflexes. enough normalized the thought was he was septic from a raging otitis (unusual w/ a fully vaccinated o/w normal kiddo) or was more fluid down than mom was letting on. however, did have a noticeable head lag when pulled to sit (abnormal for a developmentally appropriate 6mo) which bumped botulism way up-- that combined with his resp issues bought him a quick PICU transfer.

he received his $47,000 BabyBIG treatment (free to him since we're military-- the family thanks you taxpayers 🙂 ) and has since extubated and will likely make a full recovery.

--your friendly neighborhood taxpayer money spendin' caveman
 
Good learnin' case for us students. thank you. I was guessing a genetic disorder such as Tay-Sachs until the third paragraph... that's what you get for relying on your basic science courses 🙂
 
I was guessing a genetic disorder such as Tay-Sachs until the third paragraph... that's what you get for relying on your basic science courses 🙂

That is exactly where I was going. But, I'll try not to get tricked by botulism again.
 
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