Hot Seat Case #132: 9 mo M with poor feeding
Posted on: May 20, 2019, by : Mary Beth HowardRachel O’Brian, MD, INOVA Fairfax Children’s Hospital with Ioannis Koutroulis, MD, Children’s National Medical Center
HPI:
9 month old ex FT male transferred from an outside hospital for abdominal distension and decreased oral intake. Over the past 3-4 days, parents noticed the patient had become less active, weaker and more fussy. Parents felt his abdomen was more distended, but denied any fever, emesis, or diarrhea. His bowel movements had been harder and less frequent, only 1-2 per day and he had decreased oral intake. Last bowel movement was this morning and “pasty” without blood.
Parents noted the patient had been growing well and developing normally. Currently taking breast milk in addition to solids, which had been started around 6 months of age.
At the OSH, he had an abdominal x-ray which showed gaseous distension of the colon most significantly involving the sigmoid colon and transverse colon. Labs were reportedly “normal.”
ROS:
Gen: no fever; + fatigue, fussiness, and decreased PO intake
ENT: Increased clear rhinorrhea over 2 days, drooling more over the last 24 hours
Respiratory: Denies increased work of breathing or cough
Cardiovascular: Denies color change or cyanosis
Gastrointestinal: Denies vomiting, diarrhea, abdominal pain; + constipation
Genito-Urinary: Denies decreased UOP; normal wet diapers
Musculoskeletal: Denies peripheral swelling
Neurological: + limp and increased blank stares since yesterday, not interactive like normal
Dermatological: no rashes
Past Medical Hx:
Born full term by c-section
PICU admission at 3 months of age for diarrhea with significant acidosis, AKI, and transaminitis (recovered and doing well since discharge)
Medications: none
Social Hx: lives with mother and father, no other children in the home.
Allergies: none
Immunizations: UTD
PE: BP 113/79, Temp: 99.4 °F (37.4 °C), Heart Rate: 136, Resp Rate: 44, SpO2: 100 %, Weight: 7.6 kg (20th%tile)
Constitutional: Fussy but consolable, well hydrated, well perfused
Head: Normocephalic, anterior fontanelle open and flat
ENT: Moist mucous membranes, no oral lesions appreciated.
Respiratory/Chest: CTAB, no wheezes, rales, or rhonchi. No tachypnea, no grunting or retractions.
Cardiovascular: Regular rate and rhythm. No M/R/G. Brisk cap refill
Abdomen: Soft but with moderate distension, no appreciable HSM. Decreased BS
GU: normal rectal exam, no stool in the rectal vault
Neurological: Fussy but consolable, awake and interactive, moving all extremities but laying in father’s arms. Decreased tone in b/l upper and lower extremities. Strength 4/5 throughout
Skin: Warm and dry. No rash
Repeat abdominal x-ray shows decreased gaseous distension of the colon. You go back to examine the patient and attempt to PO challenge him. His tone is decreased from prior and he appears to have significant weakness despite being awake. Dad tries to give the patient a bottle of Pedialyte, but he is unable to latch and he has a weak suck. His respiratory effort is increased, but he still has clear breath sounds throughout.
Repeat vital signs: BP 88/67, HR 145, RR 60, Temp 98.1 F, SpO2 99% in RA
On re-eaxm, you notice increased WOB with subcostal retractions, lungs CTAB without wheeze, ronchi, rales.
Patient’s BG is 42. You order a D10 bolus after which BG increases to 65.
CMP: Na 139, K 4.5, Cl 109, CO2 16, BUN 5, Cr 0.4, Ca 10.5, TPr 6.2, Albumin 4.5, AST 53, ALT 24, Alk Phos 223, TBili 0.6
CBC: WBC 12.99, Hgb 12.6, Hct 38.4, Plts 361
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Stabilize: draw blood and urine for more tests before bolus; bolus glu; check for vbg, lactate and electrolytes; and support respiratory status with NC, then HFNC, then possible intubation.
Ddx: symptoms progressing over a few days-
– toxin: expect an ingestion such as alcohol to cause hypoglycemia more acutely, but repeated exposure to a drug possible but unlikely.
– botulism: symptoms and progression fit well with this history. Think hard before giving paralytic for possible intubation. Consider using higher dose Fentanyl and midazolam only.
– neuromuscular disease: onset seems fairly rapid.
– IEM: low sugar, and previous transaminitis and acidosis. Not super certain but thinking about organic acidemia and fatty acid oxidation problem. Obtain ammonia and lactate soon, as these are actionable if very high. Obtaining blood and urine for aa and oa is important for possible diagnosis, but not first priority in ED.
– CNS: head trauma with SDH could cause symptoms over days, but doesn’t quite fit as cause of low tone. CVA: would not expect symptoms to progress over days.
– NM disease: GBS ascending, which is different than overall weakness of this child. Would like to know about reflexes. Acute flaccid paralysis that has emerged in past few years: usually only one limb affected.
Plan: PICU admission given respiratory distress in a neurologically weak infant. Involve Neuro and Genetics colleagues.
I agree with Jennifer. I’ve had 2 cases of botulism that presented just like that.