Board Review: Pediatrics
A 6 week old full term male with normal birth history, mom with normal prenatal screen presents to the Emergency Deparment with hypotonia, weak cry, and weak suck. No bowel movement for 3 days which is abnormal per mom. The patient is afebrile with normal heart rate and blood pressure, respiratory exam is unremarkable and the patient is not in respiratory distress. What is the the most likely diagnosis and what is the next step in management?
A. Congenital Heart Disease - intubate and give prostaglandin
B. Inborn Errors of Metabolism - check accucheck and give D5NS at 1.5 maintenance
C. Infantile Botulism - call the California State Department for BabyBIG.
D. Sepsis - broad spectrum antibiotics and IV fluid bolus.
E. Guillen-Barre Syndrome - perform lumbar puncture to confirm diagnosis.
Answer is C: Infantile Botulism
Infantile Botulism is the most common form of botulism usually afftecting children less than 1 year old. The first symptom is often constipation but clinical manifestations can range from mild hypotonia to symmetrical descending flaccid paralysis with non-reactive pupil, weak suck, weak gag, weak cry, decreased social milestones. Some patients ultimately require respiratory support via intubation and mechanical ventilation so disposition must be PICU. Risk factors include geography (soil of California-Utah and of the Eastern Pennsylvania-New Jersey-Delaware area.), rural areas, honey, and exposure to soil from construction sites. Diagnosis is via stool specimen which takes a while as it gets sent to the CDC so if clinical suspicion is high enough, get your pediatric infectious disease and neurology specialists involved early and call California State Department for BabyBIG, an immunoglobulin to neutralize the botulinum toxin. Other aspects of emergency medicine management include airway management given chance of respiratory compromise, full sepsis work up (including lumbar puncture) and broad spectrum antibiotics, assess fluid status. D. Sepsis is not correct here but have a very low threshold to perform the full sepsis work up including Lumbar Puncture and broad spectrum antibiotics. Avoid Gentamycin when considering Infantile Botulism as it can potentiate toxin effect.
While A. Congenital Heart Disease is to be considered on the differential diagnosis of the undifferentiated sick infant, there is no indication of this given no cyanosis, no mention of diaphoresis with feeds. If you were to give Prostaglandin, give at 0.05-0.1 ug/kg/minute and intubate as 12% of patients become apneic. While B. Inborn Errors of Metabolism (IEM's) is the most common alternative diagnosis when considering botulism, the above question stem is classic botulism presentation. Consider sending IEM screening labs from the ED but do not delay BabyBIG process, sepsis work up/LP, or respiratory management. E. Guillen-Barre Syndrome (GBS) should be on the differential diagnosis. Asking about recent vaccinations and or infections may help hone in on a trigger. The Lumbar Puncture will look for albuminocytoloic dissociation. GBS is usually an ascending symmetric flaccid paralysis but the Miller-Fischer variant can be descending.