Nelly Gutierrez Martinez, MD
Pediatric Resident
University of Hawaii Pediatric Residency Program, United States
Disclosure information not submitted.
Surabhi Khasgiwala, MBBS
Pediatric Resident
University of Hawaii Pediatric Residency Program, United States
Disclosure information not submitted.
Len Tanaka, MD
Pediatric Intensivist
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Aileen Tanaka, MD
Pediatric Neurologist
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Konstantine Xoinis, MD
Pediatric Intensivist
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
John Harrington, MD
Pediatic Intensivist
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Keith Abe, MD
Pediatric Neurologist
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Rupert Chang, MD
Pediatric Intensivist
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Prashant Purohit, MD FAAP
Pediatric Intensivist
Kapiolani Medical Center for Women and Children, Hawaii, United States
Disclosure information not submitted.
Title: Botulism in an 8 week old infant: diagnostic challenges in a non-endemic area
An 8-week old female with no significant past medical history presented with decreased oral intake, cough, and grunting. She had an episode of hypoxemia, and bradycardia in the ED requiring bag-mask ventilation followed by NIPPV initiation. She also had an episode of seizure-like movements. CXR showed right upper lobe atelectasis. Her initial laboratory workup was unremarkable. She was admitted to the PICU with stable vitals on NIPPV, GCS 12, intact cough and gag reflexes, DTR 1+, and no focal abnormalities. Progressively her GCS reduced to 5, lost cough and gag reflexes, and exhibited minimal spontaneous movements; requiring emergent intubation. EEG showed mild background slowing and poor organization, abnormal temporal sharp waves, without electrographic seizures. CSF analysis for infectious and metabolic disorders, and MRI brain were unremarkable. Ammonia, carnitine/acylcarnitine profile, plasma amino acid, pyruvate, lactate, and urine organic acids were unremarkable.
Because of progressive dysphagia, facial and extremity weakness, ophthalmoparesis, mydriasis with poorly reactive pupils, and absence of other diagnoses, a botulism stool toxin was sent. She was administered botulism immune globulin (BIG) on day 4 of hospitalization. Her stool test returned positive for botulism toxin after 9 days. She required mechanical ventilation for 18 days, followed by NIPPV for 3 days, and was discharge home after 36 days.
Discussion:
Botulism is rare in 8 weeks old infant in a non-endemic area like Hawaii. There was a lack of exposure to honey, canned food, and construction site soil in our patient. The plausible mechanisms in such cases are related to clostridial colonization of large bowel and toxin production because of lack of protective bacterial flora, and clostridium-inhibiting bile.
The presence of altered mental status, right upper lobe atelectasis, seizure-like movements, and abnormal EEG added diagnostic challenges in our case. We confirmed the diagnosis of infantile botulism with positive stool toxin tests, her response to BIG, and unremarkable other workup. The results of stool toxin were not immediately available and early administration of BIG was important to reduce morbidity and mortality. High index of suspicion was important and this case report will serve as an important reminder.