Brown Pediatrics

Brown's Pediatric Residency Blog

Category: Emergency Medicine

Metabolism gone wild!

Case: Last week we talked about Jane, an otherwise healthy 2-week old girl. Let’s change the story a bit to hit on another consideration in infants. Instead of simply being febrile and fussy, let’s say that she comes back to the ED, this time afebrile, but lethargic with poor cap refill. How does our differential change now?

 

 

Image Credit: Pixabay

What are the important diagnostic considerations for neonates and infants who present very ill?

  1. Infection
  2. Metabolic/Endocrinologic
  3. Trauma
  4. Cardiac
  5. Surgical emergencies

For those of you who like acronyms, consider “THE MISFITS” in neonates and young children presenting with undifferentiated shock (adapted from post on PEM Playbook)

  • Trauma
  • Heart Disease/Hypovolemia
  • Endocrine Emergencies
  • Metabolic
  • Inborn errors of metabolism (to get this acronym to work, there may be some repetition)
  • Seizures
  • Formula problems (think too little or too much water)
  • Intestinal disasters
  • Toxins
  • Sepsis (while this is last, all very sick infants/children should be evaluated/treated for sepsis)

 

Today we will focus on the emergency management of inborn errors of metabolism (IEM), specifically at the immediate recognition and management.

 

 

Epidemiology and Etiology

IEMs are Individually rare, but more common in aggregate- 1/5000 live births for any IEM (Ewing, 2009)

Helpful to lump metabolic deficiencies into 3 broad categories (Saudabray, 2002)

  1. Disorders leading to intoxication (think urea cycle defects)
  2. Disorders involving energy metabolism (think hypoglycemia)
  3. Errors involving synthesis or catabolism of complex molecules (e.g. lysosomal storage disorders)
    • Note: Disorders in this category are rarely treatable in emergency

How do these children present?

  • Deterioration of consciousness is one of the more common presentations of IEMs (El-Hattab, 2015)
    • Other presenting features include vomiting, seizures, apnea, hepatic failure, and cardiac disease (heart failure, cardiomyopathy, arrhythmias)
  • Specific Presentation Patterns (Ewing, 2009)
    • Hypovolemia, hyponatremia, hyperkalemia: Consider adrenal insufficiency
    • Metabolic acidosis, hyperammonemia, ketotic hypoglycemia: Consider an organic acid defect
    • Encephalopathy, respiratory alkalosis, hyperammonemia: Consider a urea cycle disorder
  • Remember, “As the neonate has an apparently limited repertoire  of responses to severe overwhelming illness, the predominant clinical signs and symptoms can be nonspecific like poor feeding, lethargy, failure to thrive, etc.” (Saudabray, 2002)

What is the immediate workup? (El-Hattab, 2015)

Image Credit: Pixabay

  • Primary Workup

    • Glucose
    • Blood Gas with Lactate
    • Serum Chemistry (including BUN/SCr)
    • Urinalysis
    • Complete Blood Count (CBC) and Differential
  • Secondary Workup

    • Specific Findings from Initial Workup and/or Exam
      • Hypoglycemia: insulin, cortisol, growth hormone, β-hydroxybutryate, plasma acylcarnitine profile, plasma amino acids and urine organic acids
      • Encephalopathy: ammonia, Liver “Function” Test (sp. ALT, AST, bilirubin)
      • Suspected galactosemia: urine reducing substances

How do you stabilize? (Note: Do not wait for labs to return to begin stabilization!)

Image Credit: Pixabay

  • ABCs (patients can present altered, apneic and/or in shock)
  • Recall the 2 main categories leading to emergencies: IEMs leading to intoxication and those resulting in energy defects (Ewing, 2009)
    1. Give Energy
      • Dextrose
        • Bolus as needed to treat hypoglycemia
        • Maintenance with D10 solutions
    2. Remove Toxins
      • Make NPO
      • Intravenous Fluids
        • D10 Half-normal saline run at 1-1.5x maintenance rate
      • Hemodialysis if indicated
        • Severe Hyperammonemia (Urea Cycle defects)

Next Steps?

Consultation with a metabolic specialist is essential!

  • Will help direct further diagnostic workup
  • Will help determine if further medical interventions (medications, vitamins, cofactors, etc)

 

Summary

  1. Inborn errors of metabolism present non-specifically
  2. Always consider IEM when presented with unwell neonate or infant
  3. For critically ill presentations, IEMs can be broken down into 2 main categories: Toxin Accumulation and/or Deficient Energy
    • Emergent Treatment (following ABCs) are directed at these two issues

Faculty Reviewer: Chanika Phornphutkul, MD

References

  • El-Hattab AW. “Inborn Errors of Metabolism.” Clinc Perinatol. 2015;1-27
  • Ewing, PH et al. “Evidence-Based Management Of Metabolic Emergencies In The Pediatric Emergency Department.” Pediatric Emergency Medicine Practice. 2009;6(10)1-16
  • Horeczko, Tim. “The Undifferentiated Sick Infant.” PEM Playbook.  http://pemplaybook.org/podcast/the-undifferentiated-sick-infant/. Accessed: 5/3/2017
  • Saudabray JM et al. ” Clinical approach to inherited metabolic disorders in neonates: an overview.” Semin Neonatol. 2002;7(1)3-15

 

This is… Lumbar Puncture

Case: Jane is a 2 week-old, previously healthy, ex- full term girl who presents to the ED from her PCPs office after being found to have a temperature of 102.5 rectally. On exam, she is fussy but consolable and has an otherwise normal exam.  In addition to blood and urine studies, you plan to perform a lumbar puncture. What would be other indications and even contraindications for an LP? What are the various techniques? Should you use local anesthesia?  

 

Lumbar Puncture: The basics

Indications

  • The most common indication for lumbar puncture is to diagnose meningitis (Bonadio, 2014)
    • Other indications include diagnosing: demyelinating diseases, subarachnoid hemorrhage, or idiopathic intracranial hypertension (formerly pseudotumor cerebri)

Contraindications

  • Suspected intracranial pressure elevation
  • Clinical/Physiological Instability (hypotension, respiratory distress, status epilepticus)
  • Coagulopathy
  • Infection of overlying skin

 

Basics of setup

After discussing the case with the team, you decide that Jane has no contraindications and that it is important to rule out meningitis.  What do you need, and how do you set up?

1.  Equipment

  • Most (if not all) of your equipment will be included in a commercially available tray (Figure 1 as an example).
    • In general, you will need the following
      • Spinal needle (1.5″ or 3″ depending on the patient)
      • sterile gloves and drapes
      • Povidone-Iodine scrub
      • Monometer tune (to measure CSF pressure)
      • Sterile tubes for CSF collection

 

Figure 1: LP Tray (Picture from Bonadio, 2014)

   

2. Position 

  • In the younger child, and in those you need to measure CSF pressures, the child should be placed in the lateral decubitus position
  • In older children, the seated position can also be used (Figure 2)
  • Remember, the spinal cord ends around L2. Therefore, the needle should enter the L3/4 or L4/5 disc space
    • The L3/4 disc space will be transected by the line that connects the iliac crests (as seen in Figure 2).

 

Figure 2: LP Landmarks (Picture from Bonadio, 2014)

 

Maximizing Success

As you are gathering your materials, you begin wondering what can be done to maximize the success of your procedure.

1.)  Anesthesia

  • Topical (“EMLA”) vs local (1% lidocaine infiltration)
    • Use of local anesthetic associated with an increased odds ratio (OR = 2.2) for success (Baxter, 2006)
    • Other RCTs (Pinheiro et al, 1993; Nigrovic, 2007) found that local infiltration did not increase success, but statistically decreased the amount of struggling in infants.
      • Note: Despite not finding any differences in success rates between the two methods, it is important to note that local infiltration did not lead to decreased success (concern for a loss of landmarks, etc).

2.) Early stylet removal (“Cincinnati” Method)

  • In this method, the stylet is removed after puncturing the epidermis
    • Baxter et al found a trend towards increased success in residents employing this method, but this was not statistically significant (Baxter, 2006)
    • Nigrovic et al did find an association between leaving the stylet in and with the composite outcome of traumatic or unsuccessful lumbar puncture (Nigrovic, 2007)

Conclusion: Use an anesthetic (topical or local infiltrate) and consider removing the stylet early

 

Now that we know what we need, where we need to go, and what helps maximize success, how do we do the procedure?

 

NEJM Tutorial

https://www.youtube.com/watch?v=weoY_9tOcJQ

Another Example from EM:RAP

Video: https://www.emrap.org/

Faculty Reviewer: Jeff Riese, MD

References

  • Baxter AL et al. “Local Anesthetic and Stylet Styles: Factors Associated with Resident Lumbar Puncture Success.” Pediatrics. 2006;117(3)876-881
  • Bonadio W. “Pediatric Lumbar Puncture and Cerebrospinal Fluid Analysis.” The Journal of Emergency Medicine. 2014;46(1)141-150.
  • Nigrovic et al. “Risk Factors for Traumatic or Unsuccessful Lumbar Punctures in Children.” Annals of Emergency Medicine. 2007;49(6)762-771
  • Pinheiro JM et al. “Role of Local Anesthesia During Lumbar Puncture in Neonates” Pediatrics. 1993;91(2)379-82

 

Fluid Therapy Part II: Resuscitation Fluids

Case Continued:  Clinically, you determine that Julius (2-year-old boy with an acute diarrheal illness) needs IV fluid resuscitation. Why give fluid?

question-mark

Image courtesy of pixabay

When given for acute resuscitation (maintenance fluid will be addressed later), the goal of fluid administration is to reverse and correct circulatory insufficiency (Arikan et al, 2008).

  • In physiologic terms, fluid therapy rests aims to restore and/or increase oxygen delivery
    • Oxygen Delivery = CO x CaO2, where CO is the cardiac output (determined by heart rate and stroke volume) and CaO2 is the oxygen carrying capacity (determined by SaO2 and Hemoglobin).
    • The goal of administering fluids is to increase preload, which in turn increases stroke volume and ultimately oxygen delivery
      • THUS, each time fluid is administered, the goal is to increase stroke volume.
      • NOTE: the assessment of “fluid responsiveness” is a large topic, and will not be addressed later

Furthermore, many studies have illustrated an improvement in patient outcome with early administration of IVF (Mederios et al, 2015)- more on this later.

What to give: Theory  professor

Image courtesy of pixabay

  • Fluids are divided into 2 main categories: Crystalloids and Colloids
    • Colloids are defined as “fluids containing high molecular weight substances that remain in the intravascular compartment, thereby generating an oncotic pressure.” (Mitra et al, 2009)
      • Examples include: synthetic starches and albumin (natural)
    • Crystalloids are salt solutions of varying composition
      • In clinical practice, examples would include 0.9% saline solution, Ringer’s Lactate, and Hartmann’s solution (see Fluid Therapy: Part I for solution components)
        • Historical Note (Myburgh, 2014): “Normal Saline” was determined in the 1880s via experiments in lysing red blood cells, which indicated the amount of salt in human blood was 0.9%. Unfortunately, these studies were flawed and physiologically, human plasma is closer to 0.6% saline. Despite these findings, “normal” saline remains ubiquitous.
  • The choice of resuscitation fluid relies on Starling’s other formula, describing the determinants of fluid movement across semipermeable membranes (Myburgh, 2014)
    • The ideal solution would remain completely within the vascular space, with little extravasation into the interstitium
      • Based on this, colloids were initially thought to carry a theoretical benefit including: more rapid plasma expansion and correction of oncotic pressure (Medeiros et al, 2015)
  • Recent experiments into the glycocalyx layer of vascular endothelium have complicated this picture, indicating that in septic shock, damage to this layer contributes to vascular permeability, thereby limiting the benefits of colloids in actual practice

How does this theory play out in the real world?

In other words, in patients presenting with hypovolemia and/or shock, what fluid should be given to restore intravascular volume?

  • Colloids vs. Crystalloids
    • From our theoretical perspective, it would seem that colloids, though markedly more expensive, would be better
    • Pediatric-centered data is sparse, but studies comparing colloids and crystalloid in septic shock (usually dengue), fail to show convincing benefit of one solution over another (Mederios et al, 2015)
      • A recent meta-analysis of adult patients with sepsis indicates that synthetic colloids, specifically 6% HES, are associated with a higher rate of renal replacement therapy and mortality (Gattas et al, 2013)
      • Conversely, in adults, the use of albumin compared to crystalloid trends towards showing a 90 day mortality benefit  (Xu et al, 2014)
  • Chloride rich vs. balanced salt solutions
    • Recently, researchers have turned their attention to comparing chloride rich (e.g. normal saline) solutions with more physiologically balanced solutions (Ringer’s Lactate or Plasma-lyte)
      • These studies arose from observational studies showing (again, in adults) that hyperchloremia is associated with acute kidney injury (Suetrong et al, 2016)
        • In the recent SPLIT trial (randomized trial comparing NS and plasma-lyte), investigators failed to show any differences between the groups. In this study, the primary outcome was AKI, with secondary outcomes being use of RRT and in-hospital mortality (Young et al, 2015)
    • While such literature is sparse in the pediatric world, numerous case reports detail the association with normal saline and hyperchloremic metabolic acidosis (Skellett S et al, 2000).

Until further studies are performed, crystalloids should be used as first line therapy in fluid resuscitation in pediatrics, with attention paid to avoiding large volumes of chloride-rich fluids.

 

Now that we’ve chose a fluid, how to administer?

  • By convention, pediatric patients are given fluid boluses in 20 ml/kg aliquots. Unfortunately, the literature detailing how “20cc/kg” came to be is sparse.
  • The 2015 PALS (pediatric advanced life support) Guidelines indicate that in patients with signs of shock (diminished pulses, cool/pale/mottled skin, prolonged capillary refill, tachycardia, and altered mental status (particularly ominous), 20cc/kg should be administered over 5-10 minutes
  • In the United States, various studies have shown adherence to the PALS guidelines in children with shock is correlated with improved mortality (Carcillo et al, 2002; Han at al, 2003) and shorter length of stay (Paul et al, 2012)
  • However, the recent FEAST study (RCT Trial, population: children in sub-Saharan Africa), showed fluid bolus therapy was associated with increased mortality (Maitland et al, 2011).
    • Presently, Canadian investigators (SQUEEZE Investigators) are investigating this question (fluid sparing vs. usual care) with results of their pilot studying informing the feasibility of a multi-center trial (Parker et al, 2016).

 

Conclusions

  • Fluid, like any other intervention, has indications and contraindications
  • When deciding to give fluid boluses, determine the underlying pathophysiologic insult (sepsis vs. hypovolemia from ongoing losses) and intervene appropriately
  • Anticipate complications (fluid overload, metabolic derangements)
    • Case control studies from single institutions indicate that in the PICU setting, fluid overload is associated with higher morbidity (Sinitsky et al, 2015) and mortality (Sutawan et al, 2016).
    • Hyperchloremic metabolic acidosis with normal saline
  • Various retrospective studies show that adherence to PALS algorithm is associated with improved outcomes

 

Faculty Review: Lee Polikoff, MD

 

References

  • Arikan AA et al. “Pediatric Shock.” Signa Vitae. 2008;3(1)13-23
  • Carcillo JA et al. “Clinical practice parameters for hemodynamic support of pediatric and neonatal patients with septic shock.” Critical Care Med. 2002;30:1365-78
  • Gattas DJ. “Fluid resuscitation with 6% hydroxyethyl starch (130/0.4 and 130/0.42) in acutely ill patients: Systematic review of effects on mortality and treatment with renal replacement therapy.” Intensive Care Medicine. 2013;39(4)558-568.
  • Han YY et al. “Early reversal of pediatric-neonatal septic shock by community physicians is associated with improved outcome. Pediatrics. 2003;112: 793-99.
  • Maitland K et al. “Mortality after Fluid Bolus in African Children with Severe Infection.” NEJM. 2011;364:2483-2495.
  • Medeiros DN et al. “Colloids for the Initial Management of Severe Sepsis and Septic Shock in Pediatric Patients: A Systematic Review.” Pediatric Emergency Care. 2015;31(11)e11- e16.
  • Mitra S et al. “Are All Colloids Same (sic)? How to Select the Right Colloid?” Indian J Anaesth. 2009;53(5)592-607
  • Myburgh JA. “Fluid resuscitation in acute medicine: what is the current situation?” Journal of Internal Medicine. 2015;277; 58–68
  • Paul R et al. “Adherence to PALS Guidelines and Hospital Length of Stay.” Pediatrics. 2012;130(2):e273-280
  • Skellett S et al. “Chasing the base deficit: hyperchloraemic acidosis following 0.9% saline fluid administration.” Arch Dis Child. 2000; 83:514-516
  • Suetrong B et al. “Hyperchloremia and moderate increase in serum chloride are associated with acute kidney injury in severe sepsis and septic shock patients.” Critical Care. 2016; 20:315
  • Sutawan IB et al. “Association of fluid overload with mortality in pediatric intensive care unit.” Crit Care Shock. 2016;19:8-13
  • Young P et al. “Effect of a Buffered Crystalloid Solution vs Saline on Acute Kidney Injury Among Patients in the Intensive Care Unit.” JAMA. 2015
  • Xu JY et al. “Comparison of the effects of albumin and crystalloid on mortality in adult patients with severe sepsis and septic shock: a meta-analysis of randomized clinical trials.” Critical Care. 2014;18:702

 

 

Fluid Therapy: Part 1

splashing

Image courtesy of Pixabay, Public Domain Pictures

Fluid therapy is likely one of the most common interventions performed in pediatrics. Until recently, fluid therapy wasn’t given much thought, “reflecting the long held notion that fluid therapy is straightforward and of little consequence to the patient” (Osteermann, 2012). This post will be the first in a likely 3-part series that looks at fluids and acid-base in the care of pediatric patients. 

Case:

Julius is a 2 year-old boy, who presents to the ED with an acute diarrheal illness, which started 3 days prior (his older brother had a similar illness 1 week prior, and is now well). Initially, Julius was drinking well, however mom notes that over the last day he has stopped drinking and doesn’t appear to be making wet diapers. On your exam, you note him to be fatigued, with dry mucus membranes and vitals are significant for mild tachypnea and moderate tachycardia.  You recognize that he is hypovolemic and want to start fluids. What should you use? (NOTE: in this context, many would make the argument for NG fluids, however in the context of the post we are going to assume that this is not possible).

 

Why do we use fluids?

water-faucet

Image courtesy of Pixabay, Public Domain Pictures

  • Fluids are used for 2 main reasons (Davidson et al, 2013):
    • Maintain intravascular volume (“Fill the Tank”)
    • Maintain water and electrolyte homeostasis (e.g. hypo- vs. hypernatremia)

 

Flashback to Med School: Fluid Compartments (Davidson et al, 2013):

  • Remember that “Total Body Water” (TBW) is about 60% of lean body weight
    • Note that neonates generally have much higher TBW (~75% of body weight) and TBW decreases with age

fluid-compartments

Adapted from Davidson et al, 2013

Tonicity vs Osmolality (Khurana, 2013)

salt

Image courtesy of Pixabay, Public Domain Pictures

  • Osmolality (Osm) = moles of solute/kg of solvent
    • Depends on number of solute particles, not the specific type of particles
      • E.g. A 1 molar solution of NaCl has an osmotic concentration of 2 Osm, as NaCl will disociate into equal parts Na+ and Cl-
      • The osmolality of human intra- and extracellular fluid is 290 milliosmoles per kg (mOsm/kg)
        • This is largely determined by sodium, chloride, and bicarbonate (and to a lesser degree, glucose and urea).
  • Tonicity
    • Describes the movement of water between 2 compartments between a semi-permeable membrane (osmotic gradient)
      • In human physiology, everything is compared with that of human plasma
    • How does this apply to fluids we infuse?
      • Water will ALWAYS travel along its concentration gradient, from areas of low Osmolality to high Osmolality
        • Hypotonic fluids will result in the net influx of water into cells
        • Hypertonic fluids will draw fluids out of cells
    • As tonicity describes movement of water, it is only influenced by substances that cannot cross membrane
      • Substances that can freely cross membranes are called “ineffective osmoles” (e.g. dextrose, urea)

As such, osmolality does not equal tonicity

  • For example, the fluid D5 1/2NS is both hyperosmolar (owing to the dextrose) and hypotonic (again owing to the dextrose).

What is in the fluid we use?

 Common Fluid Choices and Their “Ingredients”

Human Body

Normal Saline (Isotonic)

Lactated Ringer’s (Isotonic)

D5 and 0.45% NaCl (Hypotonic)

Sodium 140 meq/L 154 130 77
Potassium 4 meq/L 0 4 0
Calcium 9 mg/dl 0 2.7 0
Chloride 102 meq/L 154 109 77
Lactate 0 0 28 0
Osmolality (mOsm) 298 308 273 406

 

How does one choose a fluid?

  • To choose a fluid, you must answer the question: What am I treating?
    • As mentioned earlier, this generally falls along the lines of: do I need to restore intravascular volume and/or do I need to provide daily requirements of water and electrolytes?
  • This question will be explored further on our next post, please stay tuned!

Conclusions

  • Fluid therapy is common in pediatrics, and should be approached like any other medication: Understand indications and any contraindications
  • Fluids can be classified by their osmolality (#moles solute/weight of solvent) and by tonicity (which describes the movement of water between a selectively permeable membrane)
    • Osmolality and Tonicity are related, but not equivalent. This is due to presence of “ineffective osmoles,” which are solutes than can freely cross membranes and therefore do not influence the movement of water
      • Hypotonic fluids will result in the net influx of water into cells
      • Hypertonic fluids will draw fluids out of cells
      • There will be no net movement of water with isotonic fluids

Faculty Reviewer: Lee Polikoff, MD

Sources

http://emcrit.org/pulmcrit/three-myths-about-plasmalyte-normosol-and-lr/ (for table with various [ ]’s

Davidson D et al. “Fluid Management in Adults and Children: Core Curriculum 2014.” Am J Kidney Dis. 2013; 63(4)700-

Edelson JB et al. “Intravenous Fluid Management in the Pediatric Hospital Setting: Is Isotonic Fluid the Right Approach for all Patients.” Current Treatment Options in Pediatrics. 2015; 1:90-99.

Khurana, Indu. Textbook of Human Physiology for Dental Students, 2nd Ed. Elsevier. 2013. p18.

Ostermann M. “The importance of fluid therapy: No longer an innocent bystander.” Monitor. 2012;19(6).

Just another headache?

headache

Case: John is a 6-year-old boy with a history of congenital hydrocephalus, status post ventriculo-peritoneal (VP) shunt, who presents with acute onset of headache, which is associated with nausea and vomiting. His mother is worried about a problem with the shunt. Should you be?

 

 

Answer: YES! The remainder of the post is dedicated to the ins and outs of shunts, but we cannot overstate the importance of parental insight. In one study, parental concern had a similar sensitivity and specificity as a CT scan (Kim et al, 2007)

 

A CT scan was completed on this patient (shown below), demonstrating a marked interval increase in ventricle  size, consistent with shunt failure. John was subsequently admitted to the neurosurgery service for operative revision of the shunt.

 

vp-shunt-fail

Non contrast Head CT: Axial Reformat

vp-shunt-fail-coronalNon-contrast Head CT: coronal imaging

What is a VP Shunt?

  • A VP shunts is a plastic catheter that diverts cerebrospinal fluid (CSF) from the lateral ventricles to the peritoneal cavity
    • While VP shunts are the most common, CSF can be diverted from the ventricles to the pleural cavity and the atria of the heart (VA shunt).

Why are they placed?

  • VP shunts are intended to palliate hydrocephalus, and are one of the most commonly performed neurosurgical procedures (Pietetti et al, 2007)
  • Epidemiological studies indicate the prevalence of VP shunts is 8.2/10,000 (Piatt et al, 2008). 
  • Unfortunately, VP shunts do not correct the underlying etiology of the hydrocephalus and are therefore not curative

What can go wrong with a shunt?

  • A variety of complications can occur with a VP shunt including: mechanical obstruction or over-drainage, infection, fracture, and/or migration (Piatt et al, 2008).
  • In the first year of placement, almost 40% of shunts fail (Kestle et al, 2000; Piatt et al, 2008)
    • The rates of infection (common cause of shunt failure) tend to be highest in the first 6 months of placement.

What symptoms should be concerning?

  • The most common symptoms include: nausea, vomiting, irritability, fever, or altered level of consciousness (Browd et al, 2006)
    • Specific findings and their Likelihood Ratio (Piatt et al, 2008)
      • Bulging Fontanel: 46.1
      • Decreased level of Consciousness: 26.2
      • Irritability: 13.7
  • Less common findings include: weakness, diplopia, cranial nerve palsies (CN VI especially), ataxia
  • Examination of the tract itself is very important (ventricular insertion site in skull generally traverses posterior to ear along neck). Some findings that could indicate problems:
    • Palpation of fracture
    • Erythema of tract may herald infection
    • Evidence of fluid collection over ventricular insertion site
  • Very Important to perform abdominal exam (Browd et al, 2006)
    • Abdominal Pain carries a likelihood ratio of 12.8 for failure
    • Other signs: ascites or intra-abdominal mass (pseuodocyst)

How can I evaluate a shunt?

  • Shunt Series:
    • What It Is: An anterior-posterior and lateral x-ray detailing the entire length of the catheter
    • Given poor sensitivity for shunt malfunction, authors do not recommend as first line (Boyle et al, 2015)
  • Head CT
    • Historically, the diagnostic standard for shunt malfunction
    • If enlarged ventricles are demonstrated on exam, this is consistent with shunt malfunction (Boyle et al, 2015)
    • Pros: Fast, sensitive; Con: large dose of radiation
  • FAST MRI (also known as: half-fournier acquisition single shit turbo-spin echo sequences)
    • Use single-section T2-weighted images, acquiring images in 1-4 minutes (Boyle et al, 2015)
    • Non-inferior to head CT* (Boyle et al, 2014)- Note: study not powered to compare sensitivities
    • Pro: No radiation; Con: variable access to MRI, cost (though notably less expensive than conventional MRI)

Diagnosing Shunt Malfunction

 Modality Reported Sensitivity Reported Specificity
Shunt Series 4- 26% 92-98%
Head CT 53-92% 76-93%
FAST MRI 51-59% 89- 93%
Experienced Parents 88.90% 62.20%

Adapted from Kim et al, 2007

Conclusions

  • VP shunts offer a mechanical “solution” (not cure), to patients who are otherwise unable to adequately drain CSF
  • Shunt malfunction and/or failure is common (40% fail in the first year of placement)
  • Failures can be mechanical (fracture, obstruction, dislodged) or related to infection
  • Symptoms of shunt failure are diverse and often non-specific
  • ALWAYS take parental concerns seriously!
Reviewer: Yunika Presson, CPNP (Department of Neurosurgery)

References

Blumstein H et al. “Utility of Radiography in Suspected Ventricular Shunt Malfunction.” The Journal of Emergency Medicine. 2009;36(1)50-54

Boyle TP et al. “Radiolgraphic Evaluation of Pediatric Cerebrospinal Fluid Shunt Malfunction in the Emergency Setting.” Pediatric Emergency Care. 2015;31(6)435-440

Boyle TPet al. Comparison of rapid cranial MRI to CT for ventricular shunt malfunction.”
Pediatrics. 2014;134(1)e47-e54

Browd SR et al. “Failure of Cerebrospinal Fluid Shunts: Part I: Obstruction and Mechanical Failure.” Pediatric Neurology. 2006;34(2)83-92

Kestle J et al. “Long-term Follow-up Data from the Shunt Design Trial.” Pediatric Neurosurgery. 2000;33(5)230-236

Kim TY et al. “Test Characteristics of Parent’s Visual Analog Scale Score in Predicting Ventriculoperitoneal Shunt Malfunction in the Pediatric Emergency Department.” Pediatric Emergency Care. 2007;23(8)549-552.

Piatt JH et al. “Clinical Diagnosis of Ventriculoperitoneal Shunt Failure Among Children with Hydrocephalus.” Pediatric Emergency Care. 2008;24(4)201- 210

Pitetti R. “Emergency Department Evaluation of Ventricular Shunt Malfunction: Is a shunt series really necessary?” Pediatric Emergency Care. 2007;23(3)137-141

Trouble in Paradise

upset-child

 

Case: George is a 5 year-old boy presenting to an Emergency Department (ED) complaining of abdominal pain and loose stools following a recent tropical vacation. How should we proceed? Is there any way that we could have prevented this?

What is traveler’s diarrhea?

    1. Classic Definition: ≥ 3 unformed stools in 24 hour period with nausea, vomiting, cramps, fever, blood in stool (Stauffer et al, 1990)
      • For infants and young children, some authors define diarrhea as ≥ 2-fold increase in unformed stool (Ashkenazi et al, 2016)
    2. Moderate diarrhea: 1-2 loose stools per 24 hour period
    3. Mild diarrhea: 1 loose stool per 24 hour period
    4. Duration (CDC, 2016)
      • Viral : 2-3 days
      • Bacterial: 3-7 days
      • Protozoal: weeks to months

Etiology (Ashkenazi et al., 2016)

bacteria_ecoli

Electron Microscope Image of E. Coli (Pixabay Image)

    1. Bacterial: E. Coli (ETEC, EHEC, EAEC, etc), Campylobacter jejuni, Salmonella spp., Shigella spp.,  are the most commonly seen, though Aeromonas spp. increasingly noted (CDC, 2016).
      • Of note, E. Coli (O157:H7) [associated with hemolytic uremic syndrome] has not been described in traveling children (Mackell, 2005)
    2. Viral: rotavirus, norovirus, adenovirus
    3. Parasite: Giardia (most common), Cryptosporidium, Cyclospora, entamoeba (uncommon)
    4. Etiologic agent generally identified in less than ⅓ of cases

Epidemiology

cruise-ship

    1. General incidence: 10-40% of travelers (Pitzinger B et al, 1991), though can affect up to 70% of travelers depending on the location they were traveling in (CDC Yellow Book)
      • Highest risk in Asia, Sub-Saharan Africa, and Latin America (Hagmann et al, 2010)
    2. Young children at the highest risk and manifest most severely (Ashkenazi et al, 2016)
    3. Children visiting family and/or friends are at higher risk as compared to tourists

Diagnosis

    1. Microbiologic identification is generally unnecessary
    2. If fever and colitis  think Campylobacter, Shigella, EHEC
    3. Predominance of upper GI symptoms  Giardia, isospora, cyclospora
    4. If recent antimicrobials  -> C. diff
    5. If ill, send cultures for salmonella

Role for Prevention? (Connor, 2015)

  1. Choosing food and beverages wisely while traveling has been the cornerstone of advice
    • Unfortunately, studies do not show benefit to this practice (Steffen et al, 2004)
  2. Hand hygiene very important
  3. For children older than 12 years old, bismuth subsalicylate has been shown to reduce incidence of traveler’s diarrhea by 50%
    • Inconvenient dosing: 2 tabs, four times daily
  4. Prophylactic antibiotics are not generally recommended
    • May be considered in “high-risk hosts” (e.g. immunosuppressed)

His dad asks: how should he treat this?

thirst

    1. Maintaining hydration is the most important treatment
      • Use urine output as a guide (if normal urine output, diarrheal illness is mild)
    2. If evidence of dehydration: Preferentially use oral rehydration solution (Desforges, 1990)
      • WHO solution made with: Glucose (20g/L), 3 salts (3.5g/L) [sodium chloride, potassium chloride, and sodium bicarbonate]
      • Rationale for use is intestinal co-transport of glucose and sodium
    3. Role of antibiotics
      • Warranted in severe diarrhea (>4 stools in 24 hr period, fever, blood/pus in stool)
        1. Azithromycin is treatment of choice (Ashkenazi et al., 2016)
        2. Rifaxamin for children ≥ 12 years old
        3. Fluoroquinolones (Note: not FDA approved for children)

Conclusions

      1. Diarrheal illness in children returning from travel is not uncommon
      2. Younger children at higher risk of significant morbidity
      3. Maintaining hydration is essential; utilize oral route
      4. Antibiotics not well studied, beneficial in severe cases
      5. For all traveler’s, utilize CDC’s Website to provide resources and guidance

Online Resources

  • CDC: http://wwwnc.cdc.gov/travel/

Faculty Reviewer: Michael Koster, MD

References

Ashkenazi S et al. “Travelers’ Diarrhea in Children: What have we learnt?” The Pediatric Infectious Disease Journal. 2016;35(6)698-700.

Connor BA. “Traveler’s Diarrhea.” CDC Health Information for International Travelers 2016. Ed. G. Brunette. Oxford University Press, 2015.  Print and Online

Desforges JF. “Oral therapy for Acute Diarrhea- The Underutilized Simple Solution.” NEJM. 1990; 323:891- 894.

Hagmann S et al. “Illness in Children After International Travel: Analysis From the GeoSentinel Surveillance Network.” Pediatrics. 2010. 125(5)e1072-e1080

Mackell S. “Traveler’s Diarrhea in the Pediatric Population: Etiology and Impact.” Clin Infect Dis. 2005;41(Suppl 8)S547-S552.

Pitzinger B et al. “Incidence and clinical features of traveler’s diarrhea in infants and children.” The Pediatric Infectious Disease Journal. 1991;10(10)

Stauffer WM et al. “Traveling with Infants and Small Children. Part III: Traveler’s Diarrhea.” Journal of Travel Medicine. 2002;9(3):141-50

Steffen R et al. “Epidemiology of Travelers’ Diarrhea: Details of a Global Survey.” J Travel Med. 2004;11(4)231-238.

 

 

“Water, water, everywhere…”

woman-and-baby

 

Case: Zoe is a 10 day old ex- full term female, born to a G1P0 →1 presenting with feeding difficulties. Per her mother, she is exclusively breastfed and had initially had been doing “ok” but for the last couple days, has been more sleepy than usual and not feeding as well. She also notes that during this time, her eyes have become a bit more yellow.

 

On exam, you note an infant in no distress, but she sleeps comfortably through your exam. Jaundice is appreciated. Vitals are normal, but you note she has lost 12% of her birth weight. Her HEENT is notable for a sunken anterior fontanelle. Her exam is otherwise benign. Concerned for hyperbilirubinemia and dehydration, you order a complete metabolic panel, which, among other abnormalities, is significant for a serum sodium of 165 meq/L.

 

Why is her sodium so high?

Diagnosis: Severe neonatal hypernatremic dehydration

 

Pathophysiology

  • In this case, the most likely etiology is ineffective breastfeeding (also termed lactation failure), which is a rare, but increasing cause of hypernatremic dehydration (Mortiz et al, 2002)
  • In all humans (not just neonates), hypernatremia results from one of two mechanisms: inadequate access to free water and/or an inability to concentrate urine
  • Breastfeeding failure leads to inadequate fluid intake, but is also related to the higher concentration of sodium in breast milk (Morton, 1994)

 

How do patients present? (Moritz et al, 2005)

scaleOver 70% of patients had > 10% weight loss

 

Signs at Presentation

% Of Infants (n=70)

Jaundice 81
Poor PO Intake 61
Decreased Urine Output 36
Fever 20

Table Adapted from Moritz et al, 2005

 

How common is this problem?

  • Neonatal hypernatremic dehydration is rare. A review of admissions to a major children’s hospital found that over 4 years, 1.9% of term and near term infants were admitted for hypernatremic dehydration (Mortiz et al., 2005)
  • Most commonly affects primiparous mothers

 

How should we treat?

  • The goal of treatment is to lower serum sodium in a slow and controlled fashion
  • Conventional teaching states that sodium should not be lowered faster than 0.5mEq/hr and in fact, recent studies suggest that correction faster than 0.5mEq/L/hr is independently associated with poor neurologic outcomes and seizures (Bolat et al, 2013)
  • Specifics (based on protocol detailed in Bolat et al)
    • Emergency Phase
      • Correct shock immediately (within 30 mins) with 10-20 cc/kg 0.9% saline
    • Rehydration Phase
      • Calculated Free Water Deficit
      • Composition of fluid for rehydration is dependent on serum sodium; remember, in patients with high serum concentrations, “normal saline” will be hypotonic (154 meq/L)
      • Serum sodium should be decreased by 0.5meq/L/hr over the first 24-48 hours
      • If a patient is urinating, add 40 meq potassium to fluids

 

What are the neurological outcomes?

  • In the aforementioned study (Bolat et al, 2013), researchers found that presenting serum sodium >160 meq/L was an independent predictor of mortality (OR: 1.9) and correction faster than 0.5 meq/hr was independently associated with an increased risk of seizures (OR: 4.3)
  • At 6 months of age, patients were screened with the Denver Developemental Screening Test II. Serum sodium > 165 meq/L on presentation was associated with worse outcome.

Conclusions

  • Neonatal hypernatremic dehydration is a rare complication of exclusive breastfeeding, primarily seen with primiparous mothers and  can have devastating consequences
  • Clinicians need to be aware of this complication and ensure infants  who are exclusively breastfed are followed closely to ensure adequate breastfeeding and weight gain
  • If hypernatremic dehydration is encountered, it is imperative to 1.) treat shock initially and 2.) ensure that serum sodium is NOT corrected faster than 0.5 meq/hour

Resident Reviewer: Marie Carillo, MD

References

  • Ahmed A et al. “Complications Due to Breastfeeding Associated Hypernatremic Dehydration.” Journal of Clinical Neonatology. 2014;3(3):153-157
  • Bolat F et al. “What Is the Safe Approach for Neonatal Hypernatremic Dehydration?” Pediatric Emergency Care. 2013;29(7):808-813
  • Moritz ML et al. “Breastfeeding-Associated Hypernatremia: Are We Missing the Diagnosis?” Pediatrics. 2005;116(3):e343-e347
  • Moritz ML et al. “Disorder of Water Metabolism in Children: Hyponatremia and Hypernatremia.” Pediatrics in Review. 2002;23(11):371-380
  • Morton J. “The Clinical Usefulness of Breast Milk Sodium in the Assessment of Lactogenesis” Pediatrics. 1994;93(5):802-806

Commercial Sexual Exploitation of Children (CSEC)

Post Created by: Anish Raj, MD

Case: A 15-year-old female, with a history of PTSD, presents to the ED for medical clearance after being AWOL (absent from care) from her group home for the past 3 weeks. What are critical components you should consider prior to making any disposition plans for this patient?

I.) Background (Smith, 2014)

  • Commercial Sexual Exploitation of Children (CSEC): the sexual abuse of a minor (< 18 years old) with remuneration in money, goods, or services—or the promise of money, goods, or services—to the child or a third-party for the sexual use of that child.
  • Note: CSEC is an umbrella term that encompasses child sex trafficking, escorting, survival sex, child pornography, stripping, etc.
  • Domestic Minor Sex Trafficking (DMST): the inducement of a commercial sex act of anyone under the age of eighteen by a controlling party (i.e. trafficker/exploiter/pimp) that takes place within U.S. borders and involves a child who is a U.S. citizen.
  • Note: Per the Trafficking Victims Protection Act (TVPA), in cases of child sex trafficking, the inducement of a commercial sex act of a minor (< 18 years old) is enough to meet criteria for trafficking. Force, fraud, or coercion do NOT have to be demonstrated.

 

II.) Epidemiology (IOM, 2013)

  • No consensus on estimates of incidence and prevalence
  • Most widely cited national estimate: 244,000-325,000 children are at risk for CSEC
  • Average age of initial involvement: 15 years old (Gibbs et al., 2015)
  • Rhode Island preliminary data: ~70 suspected cases over the past 3 years

 

III.) Risk Factors

  • *History of sexual abuse*: up to 70-90% of CSEC victims (Bagley & Young, 1987)
  • History of running away and/or truancy: 70% of street youth estimated to be involved in CSEC at some point (Estes & Weiner, 2001)
  • Child welfare (e.g. DCYF) involvement: 50-80% of CSEC victims (Walker, 2013)
  • Juvenile justice system (e.g. RITS) involvement
  • Adult (> 18 years old) “boyfriend”/“girlfriend”
  • Multiple sexual partners at present
  • Positive STI testing
  • Substance use

 

IV.) Physical Exam

tattoo

  • Observation: are clothing and accessories congruent with age/time/season?
  • Tattoos: name branding? explicit? gang insignia?
  • Note: In Rhode Island, individuals must be > 18 years old to receive a tattoo or piercing in a licensed parlor.
  • Signs of physical abuse (including but not limited to head trauma, oral trauma, genital trauma, and cutaneous injuries)

 

V.) Screening Examples (no brief, validated screening tool currently exists)

  • Have you or any of your friends ever exchanged sex for money, a place to stay, food, or drugs?
  • Has anyone ever asked you to have sex with someone else or made you have sex when you didn’t want to?
  • Has anyone ever taken sexual pictures of you or posted such pictures on the internet? (Greenbaum et al., 2015)

 

VI.) What To Do If You Suspect CSEC

  • Understand that it is very common for patients to NOT disclose
  • Notify Aubin Center (i.e. page on-call physician) of suspected CSEC
  • Determine need for forensic evidence collection if acute assault has taken place
  • Consult Psychiatry for evaluation
  • Confirm with law enforcement that missing persons report was filed if patient had been missing
    • If no report was filed, communicate this information to DCYF due to concern for possible neglect by caregiver
  • File report expressing concern for suspected CSEC to DCYF Hotline
  • Complete PRE without hold and fax to DCYF
  • Order screening labs: urine pregnancy, urine gonorrhea, urine chlamydia, urine trichomonas, urine toxicology screen, RPR, hepatitis C, hepatitis B, and HIV
  • Consider administration of empiric STI antibiotic treatment (e.g. ceftriaxone, azithromycin, and metronidazole)
  • Consider administration of Plan B
  • Consider administration of HIV post-exposure prophylaxis (PEP) on a case-by-case basis in coordination with Aubin Center +/- Infectious Disease team
  • Determine safe disposition plan
    • Ensure patient has an outpatient appointment at Aubin Center if being discharged
  • Consult social work and Aubin Center if patient is admitted

 

Faculty Reviewer: Christine Barron, MD

 

References:

  • Smith, Holly Austin. Walking Prey: How America’s Youth Are Vulnerable to Sex Slavery. New York: St. Martin’s, 2014. Print.
  • Institute of Medicine and National Research Council. Confronting commercial sexual exploitation and sex trafficking of minors in the United States. Washington, DC: The National Academies Press; 2013. Print.
  • Gibbs, D., Walters, J., Lutnick, A., Miller, S., & Kluckman, M. (2015). Evaluation of Services for Domestic Minor Victims of Human Tracking. Manuscript submitted for publication. Retrieved August 12, 2016, from https://www.ncjrs.gov/pdffiles1/nij/grants/248578.pdf.
  • Bagley, C., & Young, L. (1987). Juvenile Prostitution and Child Sexual Abuse: A Controlled Study. Canadian Journal of Community Mental Health, 6(1), 5-26. doi:10.7870/cjcmh-1987-0001.
  • Estes, R., & Weiner, N. (2001). The Commercial Sexual Exploitation of Children in the U.S., Canada and Mexico. Retrieved August 12, 2016 from http://www.gems-girls.org/Estes%20Wiener%202001.pdf.
  • Walker, K. (2013). Ending the Commercial Sexual Exploitation of Children: A Call for Multi-System Collaboration in California (USA, California Child Welfare Council). Retrieved August 12, 2016, from http://www.chhs.ca.gov/Child Welfare/Ending CSEC-ACallforMulti-SystemCollaborationinCA-February2013.pdf.
  • Greenbaum, J., & Crawford-Jakubiak, J. E. (2015). Child Sex Trafficking and Commercial Sexual Exploitation: Health Care Needs of Victims. Pediatrics, 135(3), 566-574. doi:10.1542/peds.2014-4138.

© 2018 Brown Pediatrics

Theme by Anders NorenUp ↑