We all are well aware of the prevalence “fever” as a presenting complaint. We are also well aware that not all “cc: fever” are created equal. Fever in a neonate may be due to a virus, but we can’t bet on it. Fever in the patient with delayed cap refill requires more contemplation than what is the maximum dose of antipyretic. Fever in a patient with sickle cell disease warrants special attention. While, generally, I have a disdain for making medical decisions based heavily on the WBC count (“WBC count is the Last Bastion of the Intellectually Destitute” – Dr. Amal Mattu @amalmattu), there are conditions that mandate respect for the WBC, like leukemia and childhood cancers. Let us take a minute to review the current recommendations for initial management of Neutropenic Fever:
Neutropenic Fever: Basics
- Neutropenia definitions can vary between institutions. [White, 2014]
- Typical definition = Absolute Neutrophil Count (ANC) < 1500 cell/microL
- Severe neutropenia is ANC < 500
- Patients receiving chemotherapy are at high risk for morbidity and mortality due to infections! [Cohen, 2016]
- The reduced immune system may lead these patients to present with limited or atypical signs of illness.
- Fever may be the only sign of significant infection.
- Neutropenic fever is one of the most common and serious complications that occurs during chemotherapy. [Lehrnbecher, 2017;Klastersky, 2016]
Low vs High Risk?
- There are a number of scoring systems used to assess a patient’s risk of bacteremia and morbidity/mortality. [Lehrnbecher, 2017]
- None of them are universally agreed upon. [Lehrnbecher, 2017; Cohen, 2016]
- There are 6 clinically based low-risk stratification strategies.
- All still require local validation. [Lehrnbecher, 2017]
- Factors known to increase risk:
- Chemotherapeutic regimen
- Advanced disease or progressive disease
- AML, ALL, Burkitt lymphoma, and relapsed acute leukemia
- History of hematopoietic stem-cell transplantation
- History of prior febrile neutropenia
- Use of high dose corticosteroids
- Presence of a focal site of infection (ex, pneumonia, abscess)
- Presence of central venous access
- Absolute Monocyte Count <100/microL
Neutropenic Fever: ED Evaluation
- While signs and symptoms may be minimal, there presence is important!
- Search for:
- Complaints of chills or rigors
- Poor Perfusion
- A Source!
- Urinary tract
- GI tract
- Perineal region infection
- Necrotizing Fasciitis (localized pain out of proportion) [Johnston, 2001]
- CNS infection
- Don’t skimp on the labs!
- CBC with differential (obviously, need this to confirm neutropenia)
- Blood cultures
- From all lumens of central line
- May also obtain peripheral culture as this can increase the detection of true bacteremia by 12%, but must be weighed against risk of contamination and pain. [Lehrnbecher, 2017]
- Cultures of other potential sources
- Urinalysis and Urine Culture, even if asymptomatic [Lehrnbecher, 2017; Sandoval, 2012]
- Basic electrolyte panel
- Chest Xray only for patients with pulmonary signs/symptoms. [Lehrnbecher, 2017; Roberts, 2012]
Neutropenic Fever: Initial Management
- DON’T JUST WAIT, RESUSCITATE!
- Again, being aware that chemotherapy may lead to subtle presentations, the patient/family may not become alert to illness until the child is much sicker.
- IV or IO boluses fluids if perfusion is poor or hypotension present.
- Empiric broad spectrum dual therapy antibiotics
- Antipseudomonal Beta-Lactam, 4th generation cephalosporin, or carbapenem PLUS a second gram-negative agent or a glycopeptide for the clinically unstable is recommended. [Lehrnbecher, 2017]
- Dual coverage is also recommended if a resistant infection is suspected. [Lehrnbecher, 2017]
- Ex: Cefepime AND Aminoglycoside AND Vancomycin
- Tailor to the patient’s prior culture results and hospital resistance patterns
- Early use of pressors if need be.
- IF THE CHILD LOOKS WELL, DON’T BE CAVALIER!
- Again, presentation can be subtle in these patients with non-functioning immune systems.
- Having a institutional protocol for evaluation and management of pediatric neutropenic fever can help expedite care and decrease time to antibiotic administration. [Cohen, 2016; Cash, 2014]
- Obtain labs, including cultures and give empiric antibiotics.
- If the child looks well, then empiric mono-therapy is recommended. [Lehrnbecher, 2017; Chuang, 2002]
- If there is a documented neutropenia in the last 24 hours, treat empirically with monotherapy antibiotics (ex, Cefepime).
- Due to the high incidence of infections with patients presenting with neutropenic fever, some advocate for giving empiric antibiotics even prior to confirmation of neutropenia in patients with: [Cohen, 2016]
- Active Chemotherapy regimens (or off less than 1 month) OR
- History of congenital or acquired neutropenia not related to chemo.
- Some children will be able to go home on oral antibiotics, but this will be contingent upon assessment of being “low risk” with the Oncologist. [Lehrnbecher, 2017]
Moral of the Morsel
- Don’t be fooled. The patient with no immune system deserves significant respect and requires our vigilance.
- Be thorough.Where is the source? Look at the mucous membranes and consider necrotizing fasciitis (i.e., look in the perineum)!
- Be aggressive! If the child looks sick, throw all of the antibiotics at them. If the child looks well, mono-therapy is recommended.
- High Risk vs Low Risk… don’t decide alone. Your physical exam and lab results will help determine whether a patient is high risk or low risk, but that determination should be made concurrently with the patient’s oncologist.
Lehrnbecher T1, Robinson P1, Fisher B1, Alexander S1, Ammann RA1, Beauchemin M1, Carlesse F1, Groll AH1, Haeusler GM1, Santolaya M1, Steinbach WJ1, Castagnola E1, Davis BL1, Dupuis LL1, Gaur AH1, Tissing WJE1, Zaoutis T1, Phillips R1, Sung L1. Guideline for the Management of Fever and Neutropenia in Children With Cancer and Hematopoietic Stem-Cell Transplantation Recipients: 2017 Update.
J Clin Oncol. 2017 May 1:JCO2016717017. PMID: 28459614
. [PubMed] [Read by QxMD]
Maertens JA1, Madero L, Reilly AF, Lehrnbecher T, Groll AH, Jafri HS, Green M, Nania JJ, Bourque MR, Wise BA, Strohmaier KM, Taylor AF, Kartsonis NA, Chow JW, Arndt CA, DePauw BE, Walsh TJ; Caspofungin Pediatric Study Group. A randomized, double-blind, multicenter study of caspofungin versus liposomal amphotericin B for empiric antifungal therapy in pediatric patients with persistent fever and neutropenia.
Pediatr Infect Dis J. 2010 May;29(5):415-20. PMID: 20431381
. [PubMed] [Read by QxMD]
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Embryology is amazing. Seriously. Think about it. Growing from one cell to all of the complex structures that form our organs is literally miraculous. It is shocking that it is able to occur correctly at all and it should be no surprise when it doesn’t go perfectly. Unfortunately, when it doesn’t go perfectly, terrible outcomes can become evident. We have previously discussed some issues when chromosomes don’t migrate properly (ex, Down’s Syndrome). We have also looked at what can happen when arteries form incorrectly (ex, AVM). Now let us take a moment to ponder the issues that can arise when an artery finds its origins incorrectly in an adjacent structure – Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA).
- Anomalous Left Coronary Artery from the Pulmonary Artery is a life-threatening congenital abnormality.
- The coronary artery, instead of originating from the aorta, arises from the pulmonary artery.
- Early on, when pulmonary vascular pressures are high, there is flow from the pulmonary artery to the myocardium.
- Later, when pulmonary vascular resistance decreases, blood is “stolen” from the myocardium and flows retrograde to the pulmonary artery.
- Collateral blood vessels can help compensate for diminished myocardial perfusion.
- Even with collateral perfusion, myocardial damage still occurs.
- LV scarring and diminished LV function
- Leads to:
- Myocardial ischemia
- Left ventricular dysfunction
- Mitral insufficiency
- Congestive heart failure
- Sudden Death
- It is a rare disease (estimates of 1 in 300,000 live births; <0.5% of congenital heart disease cases)
- Usually manifests as an isolated defect, but can be associated with other defects in ~5% of cases.
- Often asymptomatic at birth. [Levitas, 2016]
- Two primary clinical variants: [Gonzalez, 2015]
- Infants (< 2 years of age)
- Most common type (>75%)
- Poor collateral circulation
- Symptoms begin around 2 months of age, coinciding with reduction in pulmonary vascular resistance -> coronary steal -> ischemia
- Present with signs of CHF or Dilated Cardiomyopathy
- Poor feeding
- Recurrent wheezing – “Bronchiolitis”, “Asthma” [Levitas, 2016]
- Failure to Thrive [Levitas, 2016]
- Poor perfusion
- Cardiogenic shock
- ECG with evidence of ischemia
- Often anterolateral leads
- Q waves in lateral limb leads should catch your attention!
- Older Children (> 2 years of age)
- Have more substantial collateral vessels
- Clinical pictures can vary based on degree of collateral blood supply. [Levitas, 2016]
- Often are asymptomatic until late childhood or even early adulthood
- At risk for SUDDEN DEATH!
- Present with:
- Murmur (MR)
- May be only finding
- Due to ischemia of the papillary muscles
- Lack of MR diminishes likelihood of ALCAPA. [Gonzalez, 2015]
- Signs of CHF from borderline heart function worsened by acute (viral) illness
- May be initially diagnosed as myocarditis. [Levitas, 2016]
- Sudden Death (that is a terrible presenting complaint).
- ECG may be normal (that’s scary).
- ECG – looking for ischemia
- CXR – looking for cardiomegaly
- Troponin – if concern for ischemia on ECG
- Transthoracic Echo is the primary screening tool, but has it’s limitations. [Arunamata, 2015]
- Even in the setting of a normal echo, evidence of ischemia on ECG and dilated cardiomyopathy with mitral regurgitation suggests ALCAPA. [Gonzalez, 2015]
- Cardiac CT and MRI can be useful in depicting the complex anatomy and assist with planning of surgery. [Duan, 2015, Arunamata, 2015]
- Unlike other cardiomyopathies in children, the ischemic cardiomyopathy due to ALCAPA can respond to surgical therapy. [Gonzalez, 2015]
- When detected early and repaired surgically, outcomes are excellent and re-intervention is rare. [Cabrera, 2015; Gonzalez, 2015]
Moral of the Morsel
- ALCAPA is rare, but potentially devastating (like so many other pediatric illnesses). Be vigilant!
- Get the ECG for the critically ill infant and look for ischemia!
- Be wary of the infant who is being labeled as having “asthma”… may be heart failure signs.
- Listen to the older kid’s heart! (actually listen) Is there a murmur consistent with MR? Needs follow up and restricted physical activity until able to have ALCAPA ruled out.
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Parents pay a lot of attention to the skin of their child and, certainly, the skin exam is a valuable tool for the vigilant clinician (ex, Cap Refill, Petechiae below Nipple Line, Leukemia, RMSF). That being said, the majority of pediatric rashes (See Peds Rash) that present to the ED are often met with a combination of disdain and fear: “This looks like nothing, but is it something?” or “This looks awful, but is it a big deal?” One of the concerning characteristics is the target lesion, but not all target lesions are created equal. Let us take a minute to digest a morsel on Erythema Multiforme (EM).
Erythema Multiforme: Basics
- Erythema multiforme is the skin manifestations of an acute immune-mediated reaction.
- The immune-mediated reaction is often triggered by:
- Viral infection – Majority of cases.
- HSV infection
- Mycoplasma pneumonaie
- In children, medications are more closely related to SJS and TEN.
- Adults have a stronger association of EM with medications.
- Immunization [Read, 2015]
- Erythema multiforme is typically self-limited.
- Often it is considered to be on the spectrum that includes Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN), but…
- Current classification distinguish EM from this specific spectrum. [Bastuji-Garin, 1993]
- Erythema Multiforme differs from SJS, SJS-TEN Overlap, and TEN in: [Auquier-Dunant, 2002]
- Demographic characteristics
- Risk factors
- It is not associated with HIV infection, cancer, or collagen vascular diseases.
- It has less of an association with medications.
Erythema Multiforme: Mimics
- Erythema multiforme in children is often misdiagnosed. [Read, 2015; Léauté-Labrèze, 2000]
- Target lesions are NOT pathognomonic for erythema multiforme. [Read, 2015]
- There are several other conditions that need to be considered when evaluating target lesions:
- Serum Sickness
- Kawasaki Disease
- Lupus Ertyhematosus
- Urticaria multiforme
- Often mistaken for erythema multiforme. [Read, 2015]
- Acute urticaria that are annular and polycyclic wheals
- Have central clearing and ecchymotic centers
- Typically start as small macules or papule and then evolve to various sizes.
- Individual lesions fade within 24 hours.
- NOT fixed to the extremity distribution like EM is.
- Involves trunk, face, and extremities.
- Patient may also have pruritus, angioedema, and/or dermatographism (which is pretty interesting to see).
Erythema Multiforme: Diagnosis
- The classification criteria for EM are based on Bastuji-Garin, 1993.
- When observing lesions, consider location and document what you see… don’t just say “target lesions”… make note of all characteristics when able (something I am terrible at).
- EM Minor
- Epidermal detachment < 10% BSA
- Acrally distributed lesions (acral = extremities, ears, peripheral parts)
- Can be typical or raised atypical or combination of lesions
- Typical Target Lesions = <3cm diameter, symmetric, round, well-defined border, and 3 concentric color zones
- RAISED Atypical Target Lesions = <3 cm diameter, round, poorly defined border, only 2 concentric color zones.
- No mucosal involvement
- EM Major is the same as EM minor but has one or more mucosal surface involved.
- SJS and TEN
- Lesions are different:
- Flat, atypical target lesions or
- Widespread macules
- Amount of Epidermal detachment determines classification
- SJS has <10% BSA
- SJS/TEN Overlap has 10% – 30% BSA
- TEN has >30% BSA
Erythema Multiforme: Management
- Treatment is supportive.
- Symptomatic care with antipyretics and antihistamines can help.
- There have been reported concerns that NSAIDs may worsen the condition. [Dore, 2007]
- If there is a clear association with a medication, cessation of that is warranted.
- Majority of children with erythema multiforme are able to be discharged and managed as outpatients. [Read, 2015]
Moral of the Morsel
- Distribution matters. If it involves more than the extremities, think twice about calling it erythema multiforme.
- Not all target lesions are the same. Flat, atypical targets are not consistent with EM.
- Think about urticarial multiforme. Just another odd condition to keep in mind to ensure we are not misclassifying illnesses.
- Think worse first! Look for the mucosal membrane involvement closely!
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The critically ill infant and child can be “tricky” to spot sometimes. Often the phrase “That kid just doesn’t look right,” is heard around the room of critically ill children. So we recognize that “something isn’t right,” but have a hard time putting our finger on what is wrong. Often the problem stems from the fact that the blood pressure “reassures” us. We have discussed several topics related to shock previously (ex, Epi vs Dopa, Pediatric Shock Index, and Damage Control Resuscitation), but now let us focus on recognizing SHOCK and one important aspect – Capillary Refill.
Shock: Pediatric Sepsis
- Significant cause of Non-traumatic childhood mortality in the USA. [Osterman, 2015]
- When recognized early and treated aggressively, morbidity and mortality can be decreased by 50%. [Han, 2003]
- Definition of pediatric SIRS differs from adult definition in that at least one diagnostic criteria must be fever or hypothermia. [Prusakowski, 2017]
- SIRS Criteria (2 of the following, with one being fever/hypothermia)
- Fever or Hypothermia (>38.5 degrees Celsius or <36 degrees Celsius.)
- No criterion to adjust for tachycardia in the presence of fever.
- Leukocytosis or Leukopenia (abnormal for age)
- Bandemia (>10% immature neutrophils)
- Sepsis, as with adults, requires the patient have SIRS criteria AND a known or suspected infection (bacterial or viral).
- Septic Shock is Sepsis with cardiovascular dysfunction (tachycardia/bradycardia AND impaired perfusion).
Kid Physiology to Consider
- Infants and young children have a greater proportional amount of extracellular fluid to intracellular fluid compared to adults. [Prusakowski, 2017]
- Predisposes them to more risk with decreased fluid intake or excessive fluid losses.
- Young children cannot increase their myocardial contractility. [Prusakowski, 2017]
- Heart is already functioning at a high contractile state.
- In order to increase Cardiac Output, kids have to increase heart rate.
- The younger the child, though, the higher the baseline HR is, and the less likely increased cardiac output can be achieved solely with increasing the heart rate.
- This is why vasopressors and/or inotropes may be beneficial for fluid-refractory shock in kids.
- Cold Shock is more likely in children then adults.
- There is no single pathognomonic finding that defines shock.
- Hypotension is a late finding, but an ominous one, in kids.
- Constellation of findings:
- Poor perfusion
- Poor pulse quality
- Altered mental status
- Cold Shock findings:
- High Systemic Vascular Resistance
- Cold, clammy, mottled, or cyanotic extremities
- Capillary Refill > 2 seconds
- Diminished / thready pulses
- Narrow pulse pressure.
- Respect the “just ain’t right” findings:
- Poor feeding
Be Aggressive Early
- Once recognized, be aggressive within 1st hour!
- IV or IO 40-60 ml/kg of isotonic fluids PUSHED rapidly
- Optimize oxygenation
- Supplemental may be all that is initially needed.
- 30-40% of a child’s cardiac output goes to the work of breathing when critically ill, so often will require additional support (i.e., intubation).
- Broad spectrum antibiotics
- Press the Pressors!
- Fluid-refractory shock is present if the patient, after 40-60 ml/kg, is hypotensive or has poor perfusion.
- Fluid-refractory shock should be treated with vasopressors via peripheral IV or IO.
- Ideally, they would be given via central line…
- The ED is, however, NOT an ideal environment…
- So start them peripherally and plan to change to central line once time permits.
Moral of the Morsel
- Shock is difficult to recognize and requires vigilance! Pay attention to capillary refill!
- Be aggressive early! Push the fluids in, don’t hang to gravity.
- Respect what the skin is telling you! Monitor the capillary refill time. If it is still prolonged after IV boluses, treat it like refractory shock.
Dellinger RP1, Levy MM, Rhodes A, Annane D, Gerlach H, Opal SM, Sevransky JE, Sprung CL, Douglas IS, Jaeschke R, Osborn TM, Nunnally ME, Townsend SR, Reinhart K, Kleinpell RM, Angus DC, Deutschman CS, Machado FR, Rubenfeld GD, Webb S, Beale RJ, Vincent JL, Moreno R; Surviving Sepsis Campaign Guidelines Committee including The Pediatric Subgroup. Surviving Sepsis Campaign: international guidelines for management of severe sepsis and septic shock, 2012.
Intensive Care Med. 2013 Feb;39(2):165-228. PMID: 23361625
. [PubMed] [Read by QxMD]
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One of the great aspects of working in my ED is being surrounded by a multitude of amazing and brilliant teammates. One, Dr. Christyn Magill, recently provoked this Morsel of knowledge: just because it ends in “-itis” does not mean it needs antibiotics! Excellent point! We have covered this previously with Sinusitis, but another excellent example of this notion is Epididymitis!! Let us take a moment to review how Epididymitis in Children:
Acute Scrotal Pain
- Acute scrotal pain is not uncommon in the Peds ED.
- We have previously discussed some conditions that are related to scrotal pain:
- The most common concerns, however, are:
Acute Epididymitis in Children
- Epididymitis is not rare in children.
- Older literature described it as a rare condition, but newer research shows greater frequency of disease than previously known. [Nistal, 2016; Redshaw, 2014]
- Majority of cases occur in prepubertal / early adolescent boys (11-14 years). [Redshaw, 2014; Santillanes, 2011]
- Comprises up to 35-65% of all acute scrotal pain cases. [Nistal, 2016]
- Epididymitis is often considered to be due to an infection.
- Older males often have reflux of urine due to prostatic hypertrophy.
- Young males (<35 years) often have an associated sexually transmitted disease.
- What about the children who are not sexually active?
- In young children, the INCIDENCE of infection is LOW. [Nistal, 2016; Santillanes, 2011]
- Of ~1,500 patients with acute epididymitis, only ~15% had positive urine cultures. [Cristoforo, 2016]
- Unfortunately, >85% still received antibiotics. [Cristoforo, 2016; Santillanes, 2011]
- In the end, the majority are classified as idiopathic. [Redshaw, 2014; Min Joo, 2013]
- True etiology is still not fully understood.
- May be due to:
- Inflammation from adjacent torsion of appendix of testis
- Reflux of sterile urine
- Viral illness (ex, Mumps, Coxsackie B, influenza, EBV)
- Anatomic abnormalities (likely in younger patients)
- Epididymitis is usually a unilateral process. [Nistal, 2016]
- The right side is affected more often.
- Hydrocele is usually observed along with local symptoms of inflammation.
- 1st, don’t overlook the potential for torsion as the etiology!
- Distinguishing epididymitis from testicular torsion clinically can be difficult. [Redshaw, 2014]
- Have low threshold for obtaining Ultrasound.
- If history, exam, and U/S are consistent with epididymitis, consider the age:
- Young boys who are not sexually active
- Low risk for infectious etiology. [Cristoforo, 2016; Santillanes, 2011]
- May wish to treat based on abnormal urinalysis.
- Could also wait until Urine Culture results, as rates of true infections are slow low. [Cristoforo, 2016; Santillanes, 2011]
- Treat with NSAIDs, scrotal support, and rest
- Sexually active boys
- At risk for STDs!
- Consider testing and starting empiric therapy.
- Also can use NSAIDs, scrotal support, and rest (and AVOIDING SEX!).
Moral of the Morsel
- Don’t overlook torsion! Think of epididymitis as the potential cause of acute scrotal pain in young boys, but check that ultrasound!
- Just because it has “-itis” at the end of the word, does not mean it is an antibiotic deficiency!
- There is an abundance of antibiotics given to boys with epididymitis who have a low risk for infection. Don’t add to the problem. (see C. Difficile)
- Check a Urine Culture (maybe even resist the urge to react to a urinalysis) and educate the family.
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