Pediatric Leukemia/Lymphoma Presentation in the ED

• Pts most commonly present with c/o pallor or decreased activity
• Physical Exam commonly demonstrates pallor, splenomegaly, fever, hepatomegaly, lymphadenopathy, and ecchymoses/petechiae.
• CBC’s and peripheral smears are realiably abnormal
• Patients with solid tumor more commonly present with symptoms related to tumor location (ie Abd pain, Headache, etc.)

Jaffe D, Fleisher G, Grosflam J. Detection of cancer in the pediatric emergency department. Pediatr Emerg Care. 1985 Mar;1(1):11-5.

Child with a Limp

• First classify the limp:
  • Antalgic gait = shortened stance phase of the affected extremity due to PAIN
  • Trendeleburg gait = equal stance phase between involved and uninvolved side, shifted center of gravity; NOT Painful
• Etiologies
  • Painful Limp
    • 1-3 years of age: Septic Joint, Occult Trauma, Neoplasm
    • 4-10 years of age: Septic Joint, Transient Synovitis, Legg-Calve-Perthes Disease, Trauma, neoplasm, Rheumatologic D/O
    • 11 + years of age: SCFE, Rheumatologic D/O, Trauma, (consider AVN in pts with sickle cell disease)
  • Trendelenburg Gait
    • Indicative of underlying hip instability or muscle weakness
    • Think of congential hip dislocation and Neuromuscular Diseases/Disorders

Grossman, Emblad, Plantz. Orthopedic Emergencies in Pediatric Emergency Medicine Board Review.  2nd Edition. 2006. p305.

Child Abuse

• An estimated 2,815,600 children are harmed or endangered by their caretakers annually
• Fractures are among the most common injuries seen in these children and are frequently highly specific for a diagnosis of abuse.
• No fracture is pathognomonic of abuse
• Some are suggestive:
  • Spinal fx
  • Digital fx
  • Complex skull fx
  • Spiral Long Bone fx
  • Scapular fx
  • Sternal fx
  • Metaphyseal fx
  • Periosteal separation
• Some are more specific:
  • Posterior Rib fx
  • Acromioclavicular Fx
  • Multiple fxs of different ages
• Infants < 1 year of age with fractures have a high prevalence of abuse.
  
  C Y Skellern, D O Wood, A Murphy, M Crawford (2000). Non-accidental fractures in infants: Risk of further abuse. Journal of Paediatrics and Child Health 36 (6), 590–592.
  
  K. Nimkin, P. Kleinman. IMAGING OF CHILD ABUSE. Radiologic Clinics of North America, Volume 39, Issue 4, Pages 843-864
   

The Premature Infant Delivered in Your Department

An ode to my final NICU Call… just because you don’t work in a Pediatric ED, doesn’t mean you won’t encounter premature infants.

What do you need to remember when a premature infant is born in your ED (or the ambulance / cab / car)?

• Warm them and keep them warm
  • Cold stress, often overlooked, worsens acidosis and decreases surfactant function.
• Neonatal Respiratory Distress Syndrome manifests as cyanosis, tachypnea, grunting, retractions, and/or respiratory failure.
  • CXR has “ground-glass” appearance and air bronchograms
  • It is due primarily to inadequate surfactant.
  • Early administration of surfactant has proven to improve outcomes
  • Contact a neonatologist ASAP and determine if you have easy access to a surfactant product (it really is an amazing therapy).
  • You administer it down the ETT… you’ve likely intubated them by now.
• Fluids
  • Fluid Boluses are done with normal saline (10ml/kg)
  • Maintenance Fluids should be D5W or D10 (no electrolytes at first!)
• Antibiotics
  • One of the most common reasons for premature delivery is neonatal infections… don’t be stingy, start Amp/Gent (consider acyclovir) and send blood cultures at least.
     

Pierre Robin Syndrome

• The prime features of this condition are a small jaw (micrognathia), cleft palate, and posteriorly positioned tongue.
• In the newborn period, respiratory compromise from obstruction is of greatest concern.
  • Because the tongue is positioned in the back of the mouth, it tends to block the airway and cause respiratory distress.
  • In severe cases, a tracheostomy may be required to provide a stable airway for the patient. (We just had an emergent tracheostomy done in our NICU this month).
• Severity of airway obstruction varies from mild to life-threatening.
  • With only mild distress, attempt to relieve the obstruction by placing the child on his or her stomach; gravity will help to keep the tongue out of the airway.
  • Resuscitation of babies with more severe obstruction may be difficult because the micrognathia and the posteriorly protruded tongue can contribute to inadequate face-mask ventilation and make endotracheal intubation difficult (or impossible).
  • Consider LMA as a bridge to tracheostomy.
  • As soon as you recognize the presence of mirognathia, have someone call pediatric anesthesia and pediatric surgery.

Baraka, A. Laryngeal Mask Airway for Resuscitation of a Newborn with Pierre-Robin Syndrome. Anesthesiology. 83(3):646-647, September 1995.

Proteinuria

• Proteinuria on U/A may suggest underlying renal disease; however, it may be present for benign reasons as well:
  • A very concentrated urine (SG ≥ 1.020)
  • Alkaline urine (pH ≥ 7.5)
  • Presence of mucoproteins
  • Acute illness

• Benign processes almost never produce proteinuria above 1+.
• If proteinuria is detected in the ED in an asymptomatic patient:
  • Have the patient f/u with PMD for repeat u/a within 1-2 weeks
  • Recommend checking a first morning urine sample and urine protein: creatinine ratio (to rule out orthostatic/transient proteinuria).

• If proteinuria persists or is evident on first morning urine sample, then a renal biopsy may be indicated.
• Chemistry panels, CBC’s, renal ultrasound, and 24-hour urine collection rarely change the plan.
   

Chandar J, Gomez-Martin O, del Pozo R, et al. Role of routine urinalysis in asymptomatic pediatric patients.  Clin Pediatr (Phila). 2005; 44:44-48.

Hogg RJ, Portman Rj, Milliner D, Lemley KV, Eddy A, Ingelfinger J. Evaluation and management of proteinuria and nephritic syndrome in children recommendations from a pediatric nephrology panel established at the National Kidney Foundation Conference on Proteinuria, Albuminuria, Risk, Assessment, Detection, and Elimination (PARADE). Pediatrics. 2000; 105: 1242-1249.

Atrial Myxomas:

• Rare primary heart tumor
• Most involve the left side of the heart

• Symptoms may include fatigue, fever, rash, chest pain, syncope, and/or focal neuro deficits
  • Symptomatic emboli occur in 20-45% of pts with atrial myxomas
  • >50% of emboli go to the brain
  • Hemiplegia, aphasia, retinal artery occlusion, embolic “rash” in a child should all raise concern for cardiac source in pediatric pt.
    
    • Embolus from the heart is the most common cause of retinal artery occlusion in pts <40yrs.

• Emboli are most often myxoma tissue and not blood clot (so thrombolytics aren’t of much value)

Majeed Al-Mateen, et al. Cerebral Embolism From Atrial Myxoma in Pediatric Patients. Pediatrics, Aug 2003; 112: e162 - e167.

Rheumatic Fever

• Significant cause of cardiovascular morbidity in developing countries and still present in the USA, although declining in incidence.
• American Heart Association update of the Jones Criteria (1992):
  • Major Criteria
    (1)    Carditis (of any of the layers of the heart)
    (2)    Polyarthritis
    (3)    Subcutaneous Nodules
    (4)    Erythema Marginatum
    (5)    Chorea
  • Minor Criteria
    (1)    Arthralgia (not a criterion if polyarthritis is present)
    (2)    Fever
    (3)    Elevated acute-phase reactants (ESR, CRP)
    (4)    Prolonged P-R interval
• Diagnosis made by presence of TWO MAJOR or ONE MAJOR PLUS TWO MINOR.
• Diagnosis can also be made with presence of chorea and documented strep pharyngitis.

• Acute Management
  • Treat the Infection
    (1)    Penicillin (Pen V for 10 days or Pen G IM)
  • Alleviate Symptoms
    (1)    Salicylates are particularly effective for migratory arthritis
    (2)    High Dose ASA (80-100mg/kg/Day for several weeks, and then taper)
    (3)    NSAIDs for those who cannot tolerate ASA
    (4)    Steroids reserved for moderate to severe carditis.
     

Children s/p Heart Transplantation – Rejection

• Children need heart transplantation for complex congenital heart defects (hypoplastic left heart syndrome is most common) or dilated cardiomyopathies.

• Signs of Acute rejection
  • Chest Pain is uncommon
  • Common presentions: fever, myalgias, and vomiting.
    • ECG may show a decreased R wave amplitude and an increased QRS duration.
  • Labs are most often NOT diagnostic in acute rejection.
    • Troponin and CK levels may or may not be elevated.
    • Elevated LFTs are concerning for right heart failure.
  • Echo – Diastolic dysfunction is the earliest change seen in acute rejection
• Signs of Chronic Rejection
  • Clinical symptoms often related to the accelerated atherosclerosis
  • “Silent” ischemia or infarction – decreased exercise tolerance or malaise
  • Syncope

Woods, WA. Care of the Acutely Ill Pediatric Heart Transplant Recipient. Pediatric Emergency Care. 23(10):721-724, October 2007.

Severe Asthma in Pediatrics (Using “the kitchen sink” when all else fails)

Every effort should be made to avoid intubating an asthmatic pt.  Here are some possible options to consider:

• Atrovent - Multiple doses should be preferred to single doses of anticholinergics. The available evidence only supports their use in school-aged children with severe asthma exacerbation. (reference #1)
• Magnesium - Magnesium sulfate appears to be safe and beneficial in patients who present with severe acute asthma (based on 5 adult and 2 pediatric studies)
• Noninvasive ventilation - The application of NPPV in patients suffering from status asthmaticus, despite some interesting and very promising preliminary results, still remains controversial. (only one trial met criteria.  No pediatric studies)
• Heliox – No good evidence to support its use, but it is relatively safe to use, provided the patient doesn’t need more than 30% FiO2 (70%Helium)
• Ketamine – Cases suggest that for children experiencing severe asthma exacerbations, intravenous ketamine may be an effective temporizing measure to avoid exposing children to the risks associated with mechanical ventilation.
• Singulair - Intravenously administered montelukast, in addition to standard therapy, provided rapid benefits and was well tolerated among patients with acute asthma. (Study population 15yrs – 54yrs).
   

 

• References:

1. Plotnick LH, Ducharme FM. Combined inhaled anticholinergics and beta2-agonists for initial treatment of acute asthma in children. Cochrane Database of Systematic Reviews 1997, Issue 2. Art. No.: CD000060.
2. Rowe BH, Bretzlaff JA, Bourdon C, Bota GW, Camargo CA Jr. Magnesium sulfate for treating exacerbations of acute asthma in the emergency department. Cochrane Database of Systematic Reviews 1999, Issue 2. Art. No.: CD001490.
3. Ram FSF, Wellington SR, Rowe B, Wedzicha JA. Non-invasive positive pressure ventilation for treatment of respiratory failure due to severe acute exacerbations of asthma. Cochrane Database of Systematic Reviews 2005, Issue 1. Art. No.: CD004360.
4. Rodrigo, GJ. et al. Use of Helium-Oxygen Mixtures in the Treatment of Acute Asthma. Chest. 2003;123:891-896. 2003
5. T. Kent Denmark, Heather A. Crane, Lance Brown. Ketamine to avoid mechanical ventilation in severe pediatric asthma. Journal of Emergency Medicine. Volume 30, Issue 2. pages 163-166
6. James, JM. et al. A RANDOMIZED, CONTROLLED TRIAL OF INTRAVENOUS MONTELUKAST IN ACUTE ASTHMA. PEDIATRICS Vol. 114 No. 2 August 2004, pp. 547

 

Pediatric Septic Shock

• Sepsis is the most common cause of pediatric deaths worldwide.
• Recognition is paramount!  Delayed Dx = Higher Mortality
  • Hypotension is a late finding. 
  • Look for other signs of End Organ Hypoperfusion
    • Prolonged Cap Refill, Change in MS
    • Tachycardia, Tachypnea
    • Elevated Lactate / unexplained metabolic acidosis
• Management strategy is similar to that of adults
  • Get access (Don’t forget your I/O’s if necessary)!
  • Fluid Resuscitation is the most important aspect of the management
    • Get 20-60ml/kg infused within the first 15 minutes
    • Children with septic shock who get >40ml/kg before the first hour have increased survival compared to those who do not.
      • They may require 60-200ml/kg over the first few hours.
  • Get your Abx on board quickly
  • Currently there are Protocols that are based on the Adult Surviving Sepsis Campaign.

Goldstein B, Giroir B, Randolph A. International pediatric sepsis consensus conference: definitions for sepsis and organ dysfunction in pediatrics.  Pediatr Crit Care Med. 2005 Jan;6(1):2-8.

Foreign Bodies
•    No object should be left in the esophagus for >24 hrs
•    Unusual FB’s:
        ==>    Very Sharp or pointed objects may perforate the GI tract and should be removed endoscopically.
        ==>    Long objects (>6cm) or wide (>2cm) objects may not pass and should  be remove  endoscopically.
•    Button Batteries
        ==>    9% of cases involve more than one battery (x-ray mouth to anus)
        ==>    Hazards:
                    (1)    Heavy metal leakage (Mercury) – low risk but real
                    (2)    Electrical Discharge (Local tissue injury)
                    (3)    Pressure Necrosis
                    (4)    Leakage of Corrosives
        ==>    85% Pass without symptoms
                    (1)    No intervention if pass the esophagus and pt is without symptoms

•    Consider Heliox as a temporizing measure in children with respiratory distress, while awaiting endoscopy/bronchoscopy.

Concussions

• Symptoms
  • HA, Dizziness, Confusion, Tinnitus, Nausea, Vomiting, Vision changes
• 3 grades of Concussions
  • Grade 1 = transient concussion symptoms.  No amnesia.  No LOC. 
  • Grade 2 = transient concussion symptoms with amnesia.  No LOC.
  • Grade 3 = + LOC of any durations
• Return to Play Guidelines 
  • (there is no consensus statement. What follows is based on the most conservative approach)
  • Grade 1: Remove from game, Examine q 5 min.
    Return to game when asymptomatic for 20 minutes.
  • Grade 2: Remove from game until asymptomatic for 1 week.
  • Grade 3: ED evaluation.  No contact sports for 1 month once asymptomatic for 2 weeks. 
    • These apply to first concussions.  Increase concern with 2nd concussion.
• Second-Impact Syndrome
  • Occurs when a player returns to contact sport before symptoms of 1 concussion have fully  resolved.
  • Even a minor blow to the head can result in loss of brain’s autoregulation of blood flow.  
    • Leads to vascular engorgement and subsequent herniation.

Colorado Medical Society School and Sports Medicine Committee. Guidelines for the management of concussion in sports. Colo Med 1990;87:4.

Growth An infant with failure to thrive will first demonstrate poor weight gain. ==> With continued insult, there will be reduced height/length growth and then, finally, reduced head circumference growth. After birth, infants will normally loose weight initially (particularly breastfed infants). Infants should regain their birth weight by 2 weeks of life. For the first 3 months, infants should gain ~30 grams a day (~1 oz / day). By 6 months, they should have doubled their weight. By 12 months, they should have tripled their weight. By 24 months, they should have quadrupled their weight.

Henoch-Schonlein Purpura HSP is a small vessel vasculitis ==> Related to IgA nephropathy; however, IgA nephropathy more often involves young adults and predominantly affects the kidneys. Generally, HSP is a benign, self-limited disease. Pt s are NOT TOXIC appearing. ARENA (common symptoms) ==> Abdominal Pain and Vomitting (85%) +/- Bloody Stools ==> Rash (95-100%) ==> Edema (20-50%), peripheral or scrotal ==> Nephritis ==> Arthritis / Arthragias (60-80%) particularly of knees and ankles Steroids are controversial no definitive controlled trials demonstrate their efficacy.

Supracondylar Fractures Most common elbow fracture in childhood. Mechanism: Hyperextension (FOOSH) 90-95% Flexion (Fall on Flexed Elbow) 5-10% Posterior Fat Pad suggests intra-articular effusion and fracture. Complications = Ulnar, Median, and Radial nerve injuries, brachial artery injury, Volkmann s ischemic contracture, COMPARTMENT SYNDROME Associated Distal radius Fracture in ~5% of cases Palpate the wrist! Attempts in the ED at partial reduction leads to increase soft tissue injury and swelling, which will complicate the definitive reduction in the OR. -www.Wheelessonline.com

Arnold-Chiari (Chiari II) Malformation Arnold-Chiari malformation = herniation of cerebellar tissue and the medulla downwards through the foramen magnum into the upper cervical spinal canal causing compression of the upper segments of the spinal cord. Two distinct ages are identified with Chiari II malformations: infants and adolescents ==> Infants often present with Respiratory Distress, Inspiratory Stridor, and/or apnea. -- These herald impeding brainstem compromise. ==> Older children more often present with syncopal episodes or muscle weakness. Chiari Malformation needs to be considered in all children with myelomeningocele, Down s Syndrome, Hydrocephalus, Sacral Dimple, or other neurologic abnormalities presenting with respiratory distress. ==> Myelomeningocele is associated with Chiari Malformation and hydrocephalus in 80-90% of cases. Recognition is critical, since movement of the head and neck can lead to further compression of the CNS structures. Rath GP, Bithal PK, Chaturvedi A: Atypical Presentations in Chiari II Malformation. Pediatric Neurosurgery 2006;42:379-382

Congenital Heart Disease Clinical signs and symptoms of pediatric congenital heart disease are often subtle ==> Often misdiagnosed with respiratory illness or sepsis Can progress to CHF and shock ==> CHF in infants = tachypnea, tachycardia, and hepatomegaly (classic triad) -- JVD, Peripheral Edema, rales are UNCOMMON (unlike adults) Hyperoxia Test Is the etiology of the cyanosis cardiac or noncardiac? ==> If pulmonary disesase is the cause, 100% FiO2 will increase PaO2 to ~150mmHg and increase the Pulse Ox by ~10%. ==> If Heart Defect is the cause, there will be minimal improvement in condition and values. PGE1 administration ==> Used to reopen or maintain patency of ductus arteriosus until definitive intervention. ==> Consider it in a neonate presenting in shock (possibly undiagnosed ductal dependent lesion). ==> Side effects are hypotension, bradycardia, seizures, and APNEA. ==> Either intubate before or be prepared to intubate.

Lead Poisoning In Baltimore, 4.6% of kids screened had high lead levels in 2006 Plumbism presents often with vague and nonspecific symptoms; however, have high index of suspicion if: ==> Listlessness, clumsiness, or loss of developmental skills, ==> Recurrent or intermittent abdominal pain, vomiting, and constipation ==> Afebrile Convulsions ==> Resides in a house built before 1950 ==> Family history of elevated lead ==> History of Pica ==> Iron Deficiency Anemia ==> Evidence of neglect/abuse Lead Level will not come back in a timely fashion to help direct care, therefore, presumptive Chelation may be warranted. Evidence to Support Lead Posioning: ==> Micorcytic Anemia ==> Elevated Erythrocyte Protoporphyrin ==> Basophilic stippling of erythrocytes ==> Glycosuria, aminoaciduria (from development of Fanconi s Syndrome) ==> Radiopaque flecks on AXR ==> Lead Lines (dense metaphyseal bands on knee and wrist x-rays) Chelation with CaEDTA, BAL, or DMSA depending on level and symptoms.

Pediatric Hypoglycemia Hypoglycemia = <45mg/dL in symptomatic neonate; = <35mg/dL in asymptomatic Symptoms = jitteriness, tachycardia, apnea, cyanosis, tachypnea, hypotonia, temperature instability, lethargy, irritability, or abnormal cry. (almost anything!) - So check the Sugar EARLY Fasting (often from gastroenteritis and dehydration) - the most common etiology of ketotic hypoglycemia in nondiabetic kids Glucagon has diagnostic and therapeutic role If it improves hypoglycemia, then glycogen stores are sufficient. Remember to draw extra tubes for future endocrine work-up PRIOR to giving dextrose! Rule of 50 For neonates: 5ml/kg of D10; For children: 2ml/kg of D25. - 5x10=50, 2x25=50 Claudius, I., C. Fluharty, and R. Boles, The emergency department approach to newborn and childhood metabolic crisis. Emerg Med Clin North Am, 2005. 23(3): p. 843-83.