UMEM Educational Pearls - Pediatrics

Slipped capito-femoral epiphysis (SCFE) is a favorite board exam topic, and typically involves a young early or pre-adolescent obese girl with hip pain and the classic "ice cream falling off the cone" appearance on hip radiographs. However, keep these three pearls in mind when thinking about SCFE:

1. Girls > Boys, but boys may be older at presentation - don't forget 15 year old boys and SCFE.
2. An early radiographic finding may only be physis widening, so consider comparison films - the ice cream may only be levitating, but not falling off.
3. 23% of these children present with knee pain - think before diagnosing an obese 15 year old boy with a knee sprain from football. *bonus* Recall that this injury is non weight-bearing.

Show References

As RSV season approaches, remember these key points in managing bronchiolitis:

• Diagnosis is clinical - labs and XRays will not help you, unless you want to rule out a specific alternate diagnosis.  It's all about the H&P.
• Supportive care, including bulb suction of secretions, placing the child in a position of comfort, and possibly providing humidified air, is the mainstay of treatment.
  • Ribavirin, corticosteroids, and antibiotics are not indicated.  Don't use them.
  • Bronchodilators have no benefit in bronchiolitis alone, and non-response to bronchodilators supports the diagnosis of bronchiolitis.  If a trial does work, know what you are treating - some children with bronchiolitis may have an underlying component of reactive airway disease, and should be treated accordingly.
• Before disposition be sure that the child can tolerate PO.  A fussy, tachypneic child may require admission for IV hydration if they are unable to tolerate feeds - recall that infants are obligate nose breathers.
• Finally, beware the RSV bronchiolitis bounceback - the peak incidence of respiratory failure in RSV bronchiolitis is after 3-4 days of illness, when most children should be improving.

Show References

• most common cause of low platelets in children
• immune-mediated destruction of circulating platelets
• acute ITP peak incidence between 2-5 years of age; chronic ITP peaks in adolescence
• recent history (1-6 weeks) of viral infection or immunization is common
• no hepatosplenomegaly
• low platelets with megathrombocytes on smear, with normal hemoglobin (which differentiates from TTP, HUS, and DIC)
• nearly 90% of children will have normal platelet counts in 6 months
• treatment reserved for platelet counts <20,000 or significant bleeding:  IVIG (best response rate of 95%), corticosteroids (79% resposne rate), anti-rH (D) immunoglobulin (82% reesponse reate)

Show References

A common debate on the topic of pediatric burns is whether or not blisters should be debrided.  ALL PEDIATRIC BURN BLISTERS SHOULD BE DEBRIDED.  There are two reasons for this:

1. Without debridement of burn blisters, the depth of a burn cannot be assessed, and such an assessment will certainly affect treatment and disposition.
2. There is conflicting (poor) evidence that blister fluid provides both protective and damaging properties, however, there is excellent evidence that ruptured blisters, or large blisters which are likely to rupture, carry a higher risk of infection if not debrided. Therefore, all blisters should be debrided. 

The best method for debriding blisters uses sterile gauze soaked in saline, and it is important to note that pain is almost universally decreased after debridement. 

The "1, 2, 3 Ouch!" technique is exactly what it sounds like (count to three with the child, and then wipe quickly, like tearing off a bandage), and works well in older children with smaller burn areas.  Sedation may be necessary for extensive debridements, and these children may need to be taken to the OR for debridement under anesthesia.  Some burn centers utilize non-operating room anesthesia (NORA) areas for such debridements that may be prolonged or painful, but do not require the full resources of an operating room.

Show References

Pediatric Ethanol Ingestion

A young child is brought to you after accidentally drinking a shot of alcohol at a wedding party. Here is what you need to consider:

• Infants and young children who have ingested enough ethanol to cause a peak serum level ≥50 mg/dL (11 mmol/L) are at risk for profound hypoglycemia, in addition to the other effects of alcohol seen in adults The key is that the dangerous serum level is MUCH lower in children than in adults, and children require FAR smaller volume than what may be considered dangerous by adults.
• Supportive care is the key to good outcomes, with particular focus on treating hypoglycemia - check your D-sticks early and often.
• Consider child protective services involvement in every case of pediatric intoxication, and consider measurement of serum acetaminophen levels as well as other possible toxic ingestion candidates.
• Activated charcoal cannot adsorb ethanol and should only be used if other substances are being considered.
• Children who are asymptomatic for six hours, and have a safe home environment, may be discharged.

Show References

• autossomal dominant disorder most commonly in Scandinavian and British descent due to deficiency of HMB-synthetase
• most heterozygotes are asymptomatic unless some factor increases the production of pyrogens, usually medications
• common drugs include steroids, alcohol, low calorie diets, and drugs (barbituates, sulfonamide antibiiotics, grisefulvin, and synthetic estrogens (birth-control)
• attacks of abdominal pain lasting several hours is the most common symptom and may be secondary to ileus or distension, but tenderness on exam and fever are absent
• peripheral neuropathy and muscle weakness improves over days, but may take years to return to normal
• diagnose: gold standard test measures RBC HMB-synthetase, screening test of normal PBG (porphobilinogen) level in urine rules out the condition
• treatment: narcotics, IV glucose (300g/day), and IV heme (4g/day)
   

Show References

Current American Burn Association guidelines state that any child with a greater than 10% total body surface area (TBSA) burn should be admitted to a center capable of caring for pediatric burns, rather than being discharged after wound management.  However, physician use of TBSA% estimation techniques is variable.  An excellent free tool for estimating TBSA is available online, allows for automatic weight based calculation, and allows printing of your diagram.  The diagram is available at http://www.sagediagram.com/.  More to come...

Show References

Hyperpronation: This reduction technique for a nursemaid's elbow (radial head subluxation)  has been found to have better first attempt success than classic supination/flexion technique.  (Pediatrics July '98).  Support the elbow with a finger on the radial head, and forcefully hyperpronate.  

• Technique may be less painful as well.  
• Reexamine after five minutes, and normal function should be returned
• Xrays are generally unnecessary unless history and physical are not consistent with nursemaid's elbow, symptoms for greater than 12 hours, or reductions attempts are unsuccessful
• Supination/Flexion may be attempted after two failed hyperpronation attempts 

Attachments

1005212340_Hyperpronation_technique.jpg (54 Kb)

Once you've made the presumptive diagnosis of cerebral edema in Pediatric DKA (refer to part 1), here's what's next:

• DO NOT GET A HEAD CT - this will only waste your time, recall that most children with DKA have subclinical cerebral edema
• Reduce the fluid rate by at least half
• Start mannitol at 0.25-1g/kg IV over 20 minutes (may repeat in 2 hours)
• OR (not and) 3% saline at 5-10mL/kg over 30 minutes (slightly less used and supported)
• If you intubate, DO NOT HYPERVENTILATE.  A pCO2 < 22 mmHg is associated with poorer outcomes, presumably secondary to ischemia from reduced bloodflow...

Mortality from cerebral edema in DKA is 20-25%, and 15-35% of survivors have permanent disability. 

The best strategy is to do your best to avoid cerebral edema in the first place, but if you do recognize it, this is a clinical diagnosis, and you should not delay treatment for radiographic studies.

Show References

• seizure disorder occuring in infants and children <1 year of age
• mostly occur between ages 4-8 months
• classic spasm is sudden, simultaneous flexion of the head and trunk with felxion and adduction of the extremities (salaam attack Blitz-krampf)
• occurs in clusters of diminishing severity
• initiated or aggrevated by transition from sleep to wakefulness or emotions
• EEG demonstrates hypsarrhythmia: high-voltage, irregular, slow waves occuring out of synch with multiple foci
• most resolve over time without therapy, but most children have some level of mental retardation or other seizure disorder

• also known as acute cerebellitis of childhood
• most commonly affects children 2-6 years old
• about 50%  have a history of recent URI or viral GI illness
• abrupt onset of ataxia which may be mild to severe, and findings usually include hypotonia, tremor, horizontal nystagmus, and dysarthria
• child often is irritable with nausa/vomiting
• sensory exam and DTR's are normal
• CT and MRI are normal
• CSF usually demonstrates an increase in WBC, with a predemonance of lymphocytes
• 90% recover without any specific therapy in 6-8 weeks (steroids are not indicated).

EMTALA stipulates that any patient presenting to the Emergency Department is required to receive a medical screening exam regardless of age, ability to pay, or whether or not a parent accompanies the child.

EMTALA supersedes any state/local provisions or laws.

In performing a medical screening exam if an emergency medical condition exists then diagnostic testing, surgery or even transfer of hospitals may be appropriate without ever obtaining parental consent

MInors have the right to give or refuse informed assent of a procedure

If their is conflict between physician, parent or patient in the rendering of emergent care the physician must weigh the severity of the condition, risks and benefits of the treatment, as well as the patients maturity and cognition.  The physician may have to seek ethical committee review, or assistance from either social services or the court system.

If an emergent condition does not exist, EMTALA does not apply after the MSE.

Show References

• Cerebral edema develops in 20-90% of children with DKA, and mortality ranges from 20-90%
• Children younger than 5, and with newly diagnosed diabetes are at higher risk, and the risk in children in general is MUCH higher than the risk in adults
• Cerebral edema usually results from osmolarity changes during treatment, but may precede treatment
• Limit fluid repletion to isotonic fluids (Normal Saline), at a rate of no faster than 10-20 mL/kg/hr (In shock, resuscitate as usual)
• Head CT Is of limited value as the majority of children in DKA may show signs of subclinical cerebral edema, TREAT BASED ON CLINICAL SIGNS, and do not delay treatment for head CT which is likely to be abnormal in ALL kids
• Bicarb is implicated in increasing the risk of cerebral edema - focus on correction of acidosis with insulin and appropriate fluids, NOT bicarb

...more to come.

Show References

• the most common cause of stridor in the newborn
• the laryngeal skeleton is not stiff enough to resist the negative pressure during inspiration causing narrowing and stridor
• can occur at birth but most commonly seen at 2 weeks of age, and is more pronounced with agitation
• for most, close observation is sufficient as the cartilage becomes more rigid with age
• usually outgrown by 12-18 months of age
• in severe cases, feeding may be affected and nighttime obstructive hypoxia may occur

Congenital hypothyroidism (CH) is almost uniformly identified before symptoms develop because of newborn screening.  Though this problem will rarely present to the Emergency Department, it is not uncommon for parents with poor access to care to present to EDs after being notified of an abnormal screen.  Here is what you need to know:

• CH affects 1 / 3,000 live births
• When left untreated, there are many sequelae, but the most important by far is almost certain profound mental retardation
• Children treated within two weeks of birth have NORMAL intellect when followed into adolescence (compared to sibs, age matched controls)
• Children treated after two weeks have measurable declines in cognitive ability and motor skills - even though they may not develop MR, they are at VERY HIGH risk

So:

• Start treatment on ALL infants you encounter with CH, IMMEDIATELY if they are approaching 14 days of age
• Consider admission if there is any chance of a parent having poor access to prescription coverage or close followup
• Goal levels of T4 are >10 mcg/dL; infants with very low levels need IMMEDIATE TREATMENT with high dose-range levothyroxine - any delay can lead to drops of up to 20 IQ points
• Initial dose of Levothyroxine is at least 10-15 mcg/kg/day
• Tablets must be crushed and mixed with breast milk or formula, and NOT with soy, calcium or iron-containing substances which decrease levothyroxine absorption.  Liquid preparations are unreliable.

Show References

• a collection of blood UNDER the periosteum of the outer surface of the skull
• occurs in 2.5% of live births
• most commonly occurs ove the parietal bones
• because the blood is below the periosteum, it will NOT cross suture lines
• usually enlarge during the first few days of life, then slowly resolve over weeks or months
• significant bleeding is a risk
• when the blood resorbs, it can aggravate neonatal jaundice
• aspiration and xrays are not routinely indicated

Precedex (Dexmedetomidine) - Great for pediatric imaging procedures

Alpha-2 agonist with sedative properties

No analgesic effect alone, but shown to decrease the amount of opioids required for a painful procedure

Benefits pts go to sleep and awake in a more natural state.  Caregivers tend to prefer this as opposed to other sedatives.  Short recovery time- about 30 minutes

Adverse effects include bradycardia and hypotension.  Not recommended in any child with cardiac abnormalities.  Paradoxical hypertension with loading dose has also been observed

Effective for MRI or CT scans at loading doses of 2mcg/kg over ten minutes, then maintenance of 1mcg/kg/hr

Residents can gain experience with Precedex with Peds sedation on M,W,F mornings with sedation team, contact me to arrange a time for you to participate.

The umbilical site normally heals by 1 month of age. 

Any fluid draining after this period suggests an abnormal connection between the surface of the abdomen and the underlying structures, and requires further investigation.  Clear yellow fluid could represent a persistent connection of the bladder with the umbilicus called a patent urachus. The fluid that leaks is actually urine. The treatment is surgical closure of the connection.

Pus oozing from the umbilical stump would imply infection, especially if there is concomitant redness of the skin around the umbilicus.  An omphalitis can be life-threatening, and requires admission for invtravenous antibiotics.

Umbilical hernias are common in infants, and are usually noted with diastasis of the rectus muscles.  Most umbilical hernias resovle by school age, and do not require surgical intervention.

An umbilical granuloma is a small piece of bright red, moist flesh that remains in the umbilicus after cord separation. It is scar tissue, usually on a stalk, that did not become normally covered with skin cells. It contains no nerves and has no feeling. Most can be simply cauterised with silver nitrate.

Pediatric Constipation is a common presentation to PED and large percentage of GI clinic patient volume

Defined as less than 2 stools per week for two weeks with hard, large pellet like stools

Broad Differential includes functional constipation (most common), stricture, obstruction, celiac disease, Hirschsprung, hypothyroid, Cow's milk protein allergy, CF and spina bifida.  Always inspect the spine and perform rectal

Success of treatment is based on the aggressive nature of treatment and timing of treatment.  Ttreatment is longer and more difficult if patient has to wait on referral to GI specialist.

• Clean out with enema and stool softener (miralax BID for two days, followed by daily maintenance regimen is most common)
• Cheaper and effective regimens include mineral oil, kondremul or lactulose
• Encourage behavioral therapy with routine toilet time and rewards
• Increase fiber in diet to 8-10 grams for toddlers, 12-14 preschool and 14-16 for school age
• Initial treatment is safe and does not require electrolyte monitoring.
• Failed treatment and bounceback may require GI consult, inpatient Golytely therapy with electrolyte monitoring

Hyperleukocytosis is often seen in acute presentations childhood leukemias, and is defined as a WBC count of greater than 30-50K.  Complications usually arise at counts greater than 300, however, keep in mind that automated cell counters may underestimate very high white counts.

Complications include:

• Hyperviscosity Syndrome / Leukostasis
  • Risk of CVA, PE, Mesenteric Ischemia, etc.
• Tumor Lysis Syndrome (TLS)
  • Risk of fatal arrhythmia, may monitor with K, LDH, Uric Acid
• Disseminated Intravascular Coagultion (DIC)

Treatment:

• EMERGENT LEUKOREDUCTION APHERESIS, aka Leukopheresis
• This is a true emergency - if you are at a facility without leukopheresis capability, the fastest transport mode possible is indicated - fly, don't drive
• Temporizing measures include fluids, fluids, and fluids
• Allopurinol / Rasburicase may be considered, but not if this will delay transport, especially if there is no evidence of TLS - this decision may be made in consultation with the pediatric heme/onc specialist who is helping to arrange for leukopheresis