UMEM Educational Pearls - Pediatrics

• Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital defect in which there is an altered origin of the left coronary artery (also known as Bland-White-Garland syndrome)
• Generally asymptomatic at birth, but can present in late infancy, toddlerhood, or later with signs of congestive heart failure, a myocarditis picture, or sudden cardiac death
• Flow through the left coronary artery is normal at birth due to high pulmonary pressures, but as those pressures drop the blood flow drops as well and may become reversed due to the pressure gradient
• This can cause chronic myocardial ischemia, the severity of which, is dependent on collateral flow
• Most patients will also develop mitral regurgitation
• Cardiomegaly may be seen on CXR (and some patients will present with respiratory symptoms/wheezing)
• EKG findings include: findings consistent with ischemia (ST changes, q waves – specifically in the anterolateral leads), leftward axis (for age), abnormal R wave progression (loss of R wave amplitude in affected leads)
• Diagnosis can generally be made with echocardiogram (although not 100% sensitive) and the disease is generally treated with surgical repair

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This was a multistate, prospective, observational cohort of children and teachers attending in person schools in kindergarden through 12th grade where the school districs had the ability to perform contact tracing and determine primary vs secondary infections.  During the study period (6/21-12/21) 46 districts had universal masking policies and 6 districts had optional masking policies.  

Districts that optionally masked had 3.6x the rate of secondary transmission compared to universally masked school districts.  Optionally masked districts had 26.4 cases of secondary transmission per 100 community acquired cases compared to only 7.3 cases in universally masked districts.

Bottom line: Universial masking was associated with reduced secondary transmission of SARS-CoV2 compared with optional masking policies. 

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• TXA has been used for pediatric non-traumatic (surgical) bleeding with good evidence
• Currently used in around 1/3 of pediatric trauma centers based on survey data
• PED-TRAX (retrospective review of pediatric trauma admissions in a combat zone) showed an association between use of TXA and decreased mortality, with no increase in thromboembolic events
• Dosing strategies in the literature and in practice have been variable (bolus at variable dosing versus bolus + infusion)
• The TIC TOC trial was recently completed - a multicenter randomized pilot study looking at 2 dosing strategies of TXA versus placebo which demonstrated feasibility of a larger study and will hopefully serve as a model for further research to determine efficacy as well as ideal dosing

Bottom line: There is not clear evidence for efficacy, but trends are positive and the documented rates of adverse effects in this population are low. It is reasonable to give, especially in patients requiring massive transfusion or who are critically ill.

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• Use of FAST is less common in pediatric trauma than in adult trauma
• FAST in pediatric trauma has a lower negative predictive value than in adults
  • 1/3 of pediatric patients with hemoperitoneum on CT will have a negative FAST
  • Lowest sensitivity and specificity is in the under 2 years age group
• A 2017 randomized clinical trial of ~900 patients showed no difference in clinical care, use of resources, or length of stay in hemodynamically stable children who received FAST + standard trauma evaluation versus standard trauma evaluation alone
• There may be a role for FAST as a screening in patients with low suspicion for intraabdominal injury in conjunction with labs and physical exam, but this has not been fully explored

Bottom line: A positive FAST warrants further workup and may be helpful in the hemodynamically unstable pediatric trauma patient, but a negative FAST does not exclude intraabdominal injury and evidence for performing FAST in hemodynamically stable pediatric patients is limited.

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• Autism spectrum disorder and other neurodevelopmental disorders can predispose to challenging ED encounters secondary to difficulties with sensory processing and communication
• Small changes to the environment can help to reduce stress, generally by decreasing stimulation
• Use quieter areas of the ED when possible, decrease volume of alarms, and consider noise cancelling headphones or white noise if available
• Consider dimming the lights, turning the monitor/computer screen away from the patient
• Allow the patient to remain in their own clothing and consider whether restrictive items such as the monitor, pulse oximeter, and blood pressure cuff are necessary (but continue to use them when they are medically appropriate)
• Offering distraction via electronics, fidget toys, or weighted blanket (or lead apron) may help with managing stress
• Ask the patient or family which modifications would be helpful for the patient and ask child life for assistance where available

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• 2/3’s present in the neonatal period and can mimic conditions such as sepsis, gastroenteritis, and meningitis requiring careful consideration to prompt testing
• Common symptoms are poor feeding, lethargy, irritability, vomiting, and encephalopathy
• May be referred in if detected on newborn screen, but not all are tested on the newborn screen
• Should look on labs for acidosis, elevated anion gap, hyperammonemia, lactic acidosis, ketosis/ketonuria, and hyper/hypoglycemia  
• Emergent treatment includes: identification and treatment of any underlying triggers (such as infection), stopping any protein intake until situation can be clarified, providing fluids with glucose (requirements of 8-10 mg/kg/min of glucose in neonates), and genetics consultation

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Pediatric spines are elastic in nature.

SCIWORA is a syndrome with neurological deficits without osseous abnormality on XR or CT.

Many patients with SCIWORA have myelopathy.

Mechanism of injury: Most commonly caused by hyperextension or flexion. Other possible mechanisms include rotational, lateral bending, or distraction.

Population: More common in younger children. This comprises 1/3 of pediatric trauma cases that have neuro deficits on exam. 

Severity depends on degree of ligamentous injury. It can be mild to severe, and cases have the potential to be unstable. 

Management: Immobilize cervical spine and consult neurosurgery. Patients often need prolonged spinal immobilization.

If the patient is altered and an adequate neurological exam cannot be obtained, a normal CT or XR of the cervical spinal is not sufficient to rule out spinal cord injury. It is important to continue monitoring neurological status. One possible etiology is spinal cord hemorrhage, and serial exams are essential. 

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Acute facial palsy is common in children and while bell’s palsy is significant proportion, there are other more concerning etiologies that make up a number of cases. A retrospective cohort study of pediatric patients with an ED diagnosis of Bell’s palsy was done using the Pediatric Health Information System and showed an incidence of 0.3% (0.03% in control) for new diagnosis of malignancy within the 60 days following the visit at which bell’s palsy was diagnosed. Younger age increased the risk. There was also a subset of patient’s excluded for diagnosis of bell’s palsy as well as malignancy at the index visit.

These numbers are small but may be clinically significant. They likely do not warrant laboratory or imaging workup as a rule but do make a case for detailed history taking and thorough exam. Consider avoiding steroids which are used commonly but lack high quality data and may undermine later efforts at tissue diagnosis of malignancy or even worsen prognosis.

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Many types of foreign bodies may be found in a child's ear. Some examples include: beads, cotton swabs, food, insects, and button batteries. 

Patients can be asymptomatic. However, they often have otalgia, pruritus, fullness, tinnitus, hearing loss, otorrhea, or bleeding. Obtain a history of the type of foreign body, when/how it entered the ear, and if there was a prior attempt at removal. Also ask if there are foreign bodies elsewhere, such as in the nose. Perform Rinne and Weber tests before and after removing the foreign body if the child is old enough to participate. 

Delayed presentation can result in edema and otitis externa. When the foreign body is sharp, there may be damage to the tympanic membrane (TM) and ossicles. 

Consult ENT when there is suspicion of damage to TM, when hearing loss is present, or when removal is especially challenging. Spherical foreign bodies are more difficult to remove. 

Remove foreign body if it can be visualized. Wax curettes, right-angled hooks, alligator forceps, and Frazier tip suctions can facilitate removal. Avoid additional trauma due to concern for edema, bleeding, TM perforation, or distal displacement of the object. Anxiety in the child will lead to increased difficulty with removal. 

A button battery in the ear is an emergency that can result in severe damage, including TM perforation, scarring or stenosis of the ear canal, and deeper injury. Seeds such as beans or peas and other absorptive material in the ear can expand, so do not irrigate when such foreign bodies are present. Living insects should be killed with alcohol, lidocaine, or mineral oil prior to performing foreign body removal. 

After removal, reassess ear canal and TM. Some foreign bodies require removal in the operating room. If the object has been successfully removed, evaluate for otitis externa or iatrogenic injury to the ear canal, and prescribe antibiotic otic drops when needed. When TM has perforated, refer for formal audiogram. ENT follow up is recommended for all patients.  

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• Pediatric acute gastroenteritis has always been a major cause of ED visits and hospitalizations.
• Pediatric complaints of vomiting and diarrhea have been on the rise, whether it be secondary to the new Omicron-variant of COVID-19, or norovirus and rotavirus which traditionally account for nearly 60% of all cases.
• Zofran (Ondansteron) 4mg for children 4-11yo weighing greater than 40kg, and up to 8mg for those older.
• Zofran prescription at discharge was associated with reduced rate of return at 72-hours and was not associated with masking alternative diagnosis like appendicitis and intussusception.
• Oral rehydration therapy (ORT) consisting of a low osmolarity solution containing sugar and salts along with zinc has also been shown to optimize treatment and diminish return visits. ORT is available in commercial packets, pre-mixed solutions, or can be made at home with table salt and sugar.
• Bottom Line: Consider providing a prescription of Zofran along with recommendations for oral rehydration therapy consisting of a low osmolarity solution containing sugar and salts to prevent outpatient treatment failure and return visits.

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Sever Disease

• Calcaneal apophysitis – inflammation of the growth plate of the calcaneus
• One of the most common causes of heel pain in adolescents, caused by repetitive stress (overuse injury)
• Most common in those who are involved in sports, especially those with lots of running and jumping
• Symptoms are heel pain and tenderness at/underneath the heel, with possible mild swelling
• Pain is reproduced by squeezing the posterior calcaneus and standing on tip toes
• Does not require imaging for typical presentation
• Treat with reduction of activity (specifically avoid painful activities), NSAIDs, and stretching exercises

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