On this first Ped EM Morsel of the New Year (HAPPY NEW YEAR EVERYONE!), I feel like it is a great opportunity to emphasize a common theme of the Morsels: remaining vigilant and looking for the severe illness masquerading as if it were a common condition.  Unfortunately, children often will present initially with unspecific symptoms leading us to under-appreciate the true severity of their illness.  Inborn Errors of Metabolism presenting to the ED is a great example of this.  Another example is Meningococcemia.

Meningococcemia

• Relatively rare… but still leads to significant cause of infectious disease mortality worldwide!
  • Virulence of Neisseria meningitidis is 100 times that of other gram-negative bacteria.
  • In the USA, the incidence is estimated to be 0.35 cases per 100,000.
  • In developing countries, the incidence can be >500 cases per 100,000.
• Tough diagnosis
  • Approximately 50% of children with meningococcal disease are not identified during their 1st medical contact.
• Invasive Disease
  • Meningitis
    • Traditionally thought of being the predominant form of the invasive disease.
  • Bacteremia / Septicemia
    • This form has increased in the proportion of cases over the past decades.
    • Accounted for ~7% of cases in the 70-80’s, but was 36% in the 90’s.
    • Often more subtle presentation – may lead to worse outcome overall.
  • Pneumonia
    • Occurs in 5-15% of cases.
  • Less commonly:
    • Arthritis, conjunctivitis, AOM, epiglottitis, pericarditis, urethritis.
• Mortality often occurs within the 1st 24 hours of the illness.
  • While the rapid progression of the disease challenges us to diagnosis it quickly, it can also offer some assistance:
  • Observation periods can be helpful when the diagnosis is not clear.

Meningococcemia Presentation

• Unfortunately, NO LAB VALUE has been found to reliably rule-out the disease.
• The initial symptoms are often similar to any other self-limiting illnesses.
  • Fever is seen in the majority of cases (not too helpful).
  • Irritability, loss of appetite, nausea, vomiting (yup… not terribly helpful either).
  • Sore throat and URI symptoms are common.
  • Naturally, symptoms often vary based on age.
    • Younger than 5 years, less likely to report headache.
    • Teenagers often complain of a sore throat.
  • These initial symptoms often last for as long as 8 hours, but the disease rapidly progresses.
• We commonly look for the stereotypical clinical features (and should always document the presence or lack of them)
  • Lethargy
  • Toxic appearance
  • Confusion (found to have a specificity of 98.1%)
  • Overt Shock
    • Recall that kids will increase their heart rate significantly before becoming hypotensive!
    • As always, keep in mind what the goal blood pressures would be for your patient.
  • Neck Stiffness
    • Actually, not that common in acute meningococcemia, but is part of the presentation with meningitis.
    • 94.3% specificity.
  • Photophobia (96.1% specificity)
  • Purpuric Rash
    • See Morsel on Petechiae and Fever
    • Initially is subtle often with just erythema.
    • Evolves to petechiae and purpura representing microvascular thrombosis and hemorrhage.
    • Unfortunately, can progress to purpura fulminans (but that is not subtle at all).

Subtle Presentation!

• Aside from the initial symptoms that lack great sensitivity and the overt signs of badness that are hard to miss, there are also three other findings that have been associated with Meningococcemia.
• These take an active mind and vigilant clinician to detect and link to the potentially severe disease.

1. Leg Pain
   • Found to have a 94.3% specificty! That is as good as neck stiffness!
   • The patient may refuse to walk.
   • The patient may merely report thigh pain.
   • This may be due to inflammatory mediators that are being released by the immune system.
2. Cold Hands and Feet
   • Found to have a LR of 2.3
3. Abnormal Skin Color

More of the story: Most of us will pick up on the classic presentation of the patient presenting with fever, purpura, and poor perfusion; however, more commonly the patient will present with more subtle signs of illness without evidence of shock.  Being vigilant and looking for the more subtle signs and being aware of the rapid progression of the disease will help find those children who will benefit most from appropriate antibiotic therapy.

References