In this episode of PEM Currents: The Pediatric Emergency Medicine Podcast, we explore pertussis, also known as whooping cough – a disease that remains a public health challenge despite widespread vaccination efforts. We will review the clinical presentation, diagnostic strategies, management protocols, infection control practices, and vaccination updates. This episode also covers what healthcare providers need to know about post-exposure prophylaxis, respiratory precautions, and managing occupational exposures.

Learning Objectives

1. Understand the clinical progression of pertussis through its three distinct stages and identify key symptoms, including age-specific presentations in infants and older children.
2. Implement effective management strategies for pertussis, including supportive care, appropriate antibiotic regimens, and post-exposure prophylaxis for contacts and healthcare providers.
3. Promote pertussis prevention by understanding vaccination schedules (DTaP vs. Tdap), addressing vaccine hesitancy, and adhering to infection control protocols in clinical settings.

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How about a fun AI song about whooping cough?

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References

StatPearls
Lauria AM, Zabbo CP. Pertussis. [Updated 2022 Oct 7]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK519008/

AAP Pediatrics in Review
Heather L. Daniels, Camille Sabella; Bordetella pertussis (Pertussis). Pediatr Rev May 2018; 39 (5): 247–257. https://doi.org/10.1542/pir.2017-0229

UpToDate

Yeh S et al. Pertussis infection in infants and children: Clinical features and diagnosis. UpToDate. Available at: https://www.uptodate.com. Accessed December 3, 2024.

MMWR

Seither R, Yusuf OB, Dramann D, et al. Coverage with Selected Vaccines and Exemption Rates Among Children in Kindergarten — United States, 2023–24 School Year. MMWR Morb Mortal Wkly Rep 2024;73:925–932. DOI: http://dx.doi.org/10.15585/mmwr.mm7341a3

Transcript

Note: This transcript was partially completed with the use of the Descript AI

Welcome to PEM Currents, the pediatric emergency medicine podcast. As always, I’m your host, Brad Sobolewski, and today we’re talking about pertussis, a disease that is challenging clinicians and public health officials alike. Despite being vaccine preventable, Pertussis is on the rise, yet again, fueled by declining vaccination rates, waning immunity, and the fact that people can’t stop coughing on each other.

In this episode, we’ll go over clinical presentation, diagnosis management, infection control, and post exposure protocols. So pertussis, or whooping cough, is caused by Bordetella pertussis, a gram negative coccobacillus. It definitely spreads via respiratory droplets, and has no environmental or animal reservoirs, making humans the sole carriers.

The incubation period averages about 7 to 10 days, and the disease progresses through some distinct clinical stages, which I will go over in a moment. Pertussis has been recognized since the 16th century. I was not practicing medicine back then. Um, with the first documented epidemic occurring in Paris in 1578.

Bordetella pertussis was isolated in 1906 by Belgian researchers, Jules Bordet and Octave Gengou, I hopefully I pronounced them right, but they’re long gone, so they won’t be mad at me,, leading to the development of a whole cell pertussis vaccine in the 1940s. Introduction of the DTP, the diphtheria tetanus pertussis vaccine, dramatically reduced disease incidence overall.

In the 1990s, we got the acellular pertussis vaccine, the DTaP, which replaced the whole cell formulation due to concerns about some side effects. So pertussis remains endemic in many regions of the world despite vaccination efforts. During the 23 24 school year, DTaP coverage among kindergartners in the United States dropped to 92.

3%, which is below the 95 percent threshold needed for herd immunity. That is is why we’re seeing an outbreak now. This is a pretty troubling trend that began during the COVID 19 pandemic and has just gotten worse since. The exemption rate for vaccines rose to 3. 3 percent. This is the highest on record.

Non medical exemptions accounted for over 93 percent of these exemptions. And 14 states in the U. S. have reported exemption rates exceeding 5 percent. Idaho is leading at 14. 3 percent. So the implications of these declining vaccination coverage rates are significant and that’s why we’re seeing more and more outbreaks, especially putting our vulnerable populations at highest risk.

Alright, let’s get back to the clinical presentation. Wait, what’s that sound? Hold on. Coughing. Yeah, so that’s the whoop and the cough of pertussis. And I’d wager that many of you have not yet heard that clinically, so that’s why I included it on this episode. So here’s the stages of disease. First is the catarrhal stage, which lasts one to two weeks.

You have rhinorrhea, mild cough, and a low grade fever, if any. You are highly contagious during this phase, but it’s often unrecognized as pertussis. Then, in the next two to eight weeks, you have the paroxysmal stage. You have these severe paroxysms of coughing, the inspiratory whoop right beforehand, post tussive emesis.

Infants, especially under six months of age, may present atypically with just apnea, cyanosis, or bradycardia. for that. Following that, you have the convalescent stage, which lasts weeks to months. You have gradual resolution of symptoms, though residual cough may persist. That’s why they call it the 100 day cough.

Aside from coughing forever, there’s some important complications you need to be aware of. And they can be severe, especially, as I noted earlier, in young infants. So respiratory complications include apnea, secondary bacterial pneumonia, and pulmonary hypertension. Children encephalopathy, often due to hypoxia.

And the mechanical complications can include rib fractures, subconjunctival hemorrhage, and even rectal prolapse due to intense coughing and valsalva. Greater than 50 percent of kids under 12 months of age with pertussis could require hospitalization. 50 percent of those kids will have apnea, 20 percent will have pneumonia, and up to 1 percent will die.

Encephalopathy occurs in about 20 percent of mortality cases, probably due to hypoxia, or maybe the toxin produced by the bacteria itself. So, making the diagnosis of pertussis starts with high index of clinical suspicion. Early diagnosis, as you’d suspect, is critical to limiting disease spread and initiating treatment.

So, PCR testing, which is widely available now, has high sensitivity in the first three to four weeks and is the preferred diagnostic test. Culture is the old gold standard, but it’s slower and less sensitive. It can take up to a week to grow. CBC might show significant lymphocytosis, um, most often in infants, but it ain’t going to make the diagnosis of pertussis for you.

And a chest x ray will just show you some non specific findings, such as peribronchial thickening in severe cases. And unless you’re worried about concomitant bacterial pneumonia, you probably don’t need a chest x ray to make the diagnosis of pertussis. You can get an isolated pertussis PCR, or Or it can come as part of a respiratory panel.

But remember those comprehensive viral respiratory panels cost 1, 600. So if you’re just worried about pertussis, don’t get the whole panel. So management starts with supportive care. Infants with apnea, cyanosis, or feeding difficulties should obviously be admitted to the hospital. They may need oxygen and or nutritional support.

And you definitely have to watch those kids very closely for the complications such as hypoxia and secondary infections. Remember, a tiny baby with pertussis can go apneic at a moment’s notice even without a persistent cough. Antibiotics reduce transmission. But do not significantly alter disease progression once the paroxysmal stage begins.

So again, you are treating with antibiotics to prevent more people from getting sick, more so than shortening the duration of illness. The main antibiotic that we use is azithromycin. For infants under 6 months of age, that’s 10mg per kg daily for 5 days. For children older than 6 months of age, 10mg per kg, max of 500mg on day 1, followed by 5mg per kg per day, max of 250mg on days 2 through 5.

That is the same dosing that you can give to a grown up. An alternative treatment, you would be trimethoprim sulfamethoxazole for patients who are allergic to macrolides. Post exposure prophylaxis is recommended for household contacts, so the people that the index patient lives with, any high risk individual, and infant, pregnant women, or immune compromised individuals that have been in any sort of contact with the person with pertussis, and and a health care worker exposed without appropriate PPE.

Again, pertussis spreads through respiratory droplets. So this necessitates strict infection control. So that starts in triage. So if you think that a patient has pertussis, then they need to be place on droplet precautions as soon as they are assessed. You wear a surgical mask and eye protection, so goggles or a face shield, and you want to maintain these precautions for five days after starting effective antibiotics or for 21 days if the patient is untreated.

As a clinician, Just ask yourself, did you wear appropriate PPE, mask and goggles? Don’t get lazy. Was the exposure prolonged or close? And rely on infection control in your institution to help decide whether or not you need post exposure prophylaxis. If you’re vaccinated and you wore PPE, you don’t need anything.

Unless you have symptoms. If you’re vaccinated and you did not wear PPE, then prophylaxis is recommended. If you’re unvaccinated and not up to date, well then what are you doing in healthcare? And immediate prophylaxis and vaccination update are required. And, okay, ’cause I just mentioned it. Let’s talk about vaccines.

So first I wanna talk about DTaP, dt, lowercase a uppercase p and t dap. Uppercase T D A P. So DTAP contain higher concentrations of diphtheria and pertussis antigens. It’s used for children under seven years of age. TDAP contains lower antigen concentrations and it’s designed for adolescents and adults to reduce reactogenicity.

There is no standalone pertussis vaccine. I’ve had patients say, well, I don’t want tetanus. Just give me the pertussis one. Well, tough Schenectes. We do not have a pertussis vaccine. alone. It’s only available in combination with diphtheria and tetanus toxoids, DTaP or Tdap. The combined vaccine boosts efficacy and ensures broader protection against all of the included infections.

Now the routine vaccination schedule, which if you are a pediatric resident, you know, like the back of your hand, the DTAP is administered at 2, 4, 6, and then between 15 and 18 months with a booster at 4 to 6 years. The Tdap is one dose at 11 to 12 years and then during every pregnancy to confer passive immunity to the newborns.

And again, depending on when you’re listening to this, you may be in the midst of a pertussis outbreak. And if you listen to this a few years later, after the original publication date in the fall of 2024, and you’re seeing another pertussis outbreak, well, dang it, we haven’t done our job. We need to strengthen school vaccination requirements.

We need to educate parents about vaccine safety and the risks of exemptions. And we need to broadly improve and ensure access to vaccinations through our community clinics. Thanks. Alright, so that’s it for this episode on Pertussis, which remains a significant public health challenge due to its severe complications in young patients and the ongoing decline in vaccination coverage.

Healthcare providers play a vital role in diagnosing and managing it, preventing its spread, and educating patients and families about the benefits of vaccination. Infection control practices and post exposure protocols are critical for protecting both clinicians and close contacts and other exposures.

Thank you so much for listening to this episode. I hope you found it educational and informative. If there’s other topics that you want to hear about, let me know. I’m on X, I’m on Blue Sky, I’m on Mastodon, I take emails, you can leave a comment on the blog, you can leave a review on your favorite podcast site, any feedback is good feedback, and encourage your colleagues to listen, and as the kids say, like and subscribe, I told my 12 year old I would say that at the end of the episode.

For PEMCurrents, the Pediatric Emergency Medicine Podcast, this has been Brad Sobolewski, see you next time.

In this episode of PEM Currents: The Pediatric Emergency Medicine Podcast, I explore the complexities of gastroesophageal reflux (GER) and gastritis in children and adolescents. I’ll make the important distinction between gastritis – which is diagnosed only via endoscopy – and dyspepsia, the term best used to describe the symptoms many patients experience. I’ll dive into the latest clinical practice guidelines and discuss evidence-based approaches to diagnosis and treatment.

Topics covered include:

• The pathophysiology of GER and GERD in the pediatric population.
• Understanding dyspepsia and its clinical presentation.
• Diagnostic strategies and when to consider further evaluation.
• The role of lifestyle and dietary modifications in management.
• Pharmacological interventions, including the use of proton pump inhibitors (PPIs), H2 blockers, and antacids.
• Current controversies and updates in pharmacological treatments.
• Management of gastritis and the consideration of Helicobacter pylori infection.

Join me as I scope out the nuances of gastroesophageal reflux and gastritis and provide practical insights for clinicians in the emergency setting.

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References

Rosen R, Vandenplas Y, Singendonk M, et al. Pediatric Gastroesophageal Reflux Clinical Practice Guidelines: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2018;66(3):516-554. doi: 10.1097/MPG.0000000000001889

Lightdale JR, Gremse DA; Section on Gastroenterology, Hepatology, and Nutrition. Gastroesophageal Reflux: Management Guidance for the Pediatrician. Pediatrics. 2013;131(5):e1684-1695. doi: 10.1542/peds.2013-0421

Tighe M, Afzal NA, Bevan A, et al. Pharmacological Treatment of Children with Gastro-oesophageal Reflux. Cochrane Database Syst Rev. 2014;2014(11):CD008550. doi: 10.1002/14651858.CD008550.pub2

Sintusek P, Mutalib M, Thapar N. Gastroesophageal Reflux Disease in Children: What’s New Right Now? World J Gastrointest Endosc. 2023;15(3):84-102. doi: 10.4253/wjge.v15.i3.84

Transcript

Note: This transcript was partially completed with the use of the Descript AI

Welcome to PEMCurrents, the pediatric emergency medicine podcast. As always, I’m your host, Brad Sobolewski, and today’s episode will focus on gastro esophageal reflux and gastritis in children and adolescents. Both conditions can present with similar symptoms. They do, though, have distinct pathophysiologies and management strategies.

So we’re going to discuss the evidence based approaches to diagnosis and treatment, and I will talk about why it is presumptive to call things gastritis before definitive diagnosis, even though I put it in the title of the episode. Alright, let’s scope things out. I’m going to begin by making a brief distinction between gastroesophageal reflux and gastroesophageal reflux disease.

So, reflux itself is a common physiologic process, especially in infants, that usually resolves by 12 to 18 months, when the gastroesophageal sphincter gets tighter, and kids spend more of their life on it. upright. Gastroesophageal reflux disease is when the patients have severe symptoms that persist and cause long term issues.

Now, either way, this is due to reflux of the stomach contents into the esophagus. Now, gastroesophageal reflux is incredibly common in infants with up to 50 percent of b cells. babies under three months regurgitating daily. This typically peaks at about four months of age and improves as the infant grows.

Gastroesophageal reflux disease is less common, but still affects about 10 percent of children and up to 10 to 20 percent of adolescents. Adolescents with GERD often present similarly to adults with heartburn and regurgitation as the primary complaints. Now there are some risk factors for GERD.

Gastroesophageal reflux and reflux disease in children. A main one is neurological impairment. So kids with cerebral palsy or other neurologic disorders have delayed gastric emptying and poor esophageal motility, increasing the risk of GERD. Children with respiratory conditions and chronic lung disease like asthma are more prone to GERD as well.

And reflux itself can exacerbate the existing respiratory symptoms either through microaspiration or vagal mediated bronchospasm. Prematurity and congenital conditions like esophageal atresia also obviously increase the risk. So gastroesophageal reflux and reflux disease result from transient relaxation of the lower esophageal sphincter.

That allows acidic gastric contents to flow back into the esophagus. Now, the lower esophageal sphincter is supposed to prevent this reflux from happening. In GERD, the sphincter relaxes too frequently or incompletely, which can lead to symptoms building up over time. Other factors like delayed gastric emptying and abnormal esophageal motility can worsen problems in general.

And the presentation for GERD varies by age. Infants will have frequent spitting up or vomiting after feeds, irritability during or after feeds, especially in the more significant cases where the esophagus is irritated. Uh, infants may start to refuse to feed due to the discomfort. They can have poor weight gain or even failure to thrive in more severe cases.

And they can have Sandefur syndrome, which is the arching of the back and dystonic posturing that occurs during or after feeds as a response to discomfort from acid reflux. This is sometimes misidentified as seizures. In older children and adolescents, heartburn is the most common symptom. It’s a burning sensation in the chest or epigastrium, and that is the classic symptom in this age group.

You can also see regurgitation, acid or food regurgitating into the mouth, leading to a sour taste. uh, ill defined epigastric or chest pain, and it’s often worse by eating or lying down after meals. And some other symptoms that you gotta think about in reflux, chronic cough, hoarseness, or even asthma like symptoms.

These extra esophageal manifestations can occur due to irritation of the upper airways by the gastric contents. Now, in the pediatric emergency department, the diagnosis of reflux is primarily clinical. You just got to take a good history and do a physical examination, especially when the symptoms are typical, like regurgitation, heartburn, or epigastric pain in an older child or adolescent.

You want to take a good history on feeding or dietary habits. In infants, you want to know the amount and frequency of feeding, especially because baby stomachs are small and no newborn stomach can hold six ounces. So, the parents may say, well, he Doesn’t ever seem full. Well, baby doesn’t know how to be full.

So really, you need to learn how much a baby can take and follow weight gain appropriately. Overfeeding a baby will lead to increased spitting up and reflux. You should also ask about older children and adolescents eating trigger foods like spicy things, caffeinated beverages, and acidic beverages like pop.

These can all exacerbate reflux. You want to ask about nocturnal symptoms. So if the kid has symptoms that are worse at night or immediately upon waking up, this may indicate reflux exacerbated by lying down, especially after meals. Red flags that you should always ask about include hematemesis, melana, dysphagia, and Unintentional weight loss, these all need further investigation for complications like esophagitis or another diagnosis such as EOE or peptic ulcer disease.

If any of those red flags are present, you should definitely consider a further diagnostic workup. Again, otherwise the diagnosis is based on history and physical examination. So if you see failure to thrive or poor weight gain, again, vomiting blood or passing black tori stools, signs of esophagitis such as painful swallowing or difficulty swallowing.

They need more workup often via GI. So diagnostic testing centers around endoscopy. That’s the gold standard and ultimately getting a camera in there. Taking a look for strictures or ulcers and taking a biopsy of the esophageal and gastric mucosa will make the diagnosis and evaluate for other things like eosinophilic esophagitis or helicobacter pylori infection.

Also get a pH monitoring and impedance probe. This measures both acid and non acid reflux events. It’s kind of like a Holter monitor for reflux. This is obviously ordered by a gastroenterologist, but But the probe itself measures reflux, and then the patient can press a button for an event monitor. And this is particularly useful in children with atypical symptoms or extra esophageal manifestations like cough or asthma, and especially in those that have had a normal endoscopy.

Contrast radiography, like a barium swallow, has much lower sensitivity and specificity than scopes or, in certain situations, even impedance probes and pH monitors. Now, that being said, imaging can be helpful if you suspect hypertrophic pyloric stenosis or other causes of obstruction such as duodenal atresia or antral webs.

But again, you should suspect these based on a detailed history and physical. A management of gastroesophageal reflux is largely dependent on the severity of symptoms and the patient’s age. And most cases of GERD in a pediatric ED can be managed conservatively with feeding or lifestyle modifications and short term pharmacotherapy.

So in infants, you want to recommend smaller, more frequent feeds. Again, overfeeding is a common contributor to reflux in infants. Maybe parents will have to thicken the feeds, but I do not recommend this in the emergency department. Um, and this practice adds rice cereal to the formula or express breast milk that may reduce regurgitation.

Again, this should only really be done under supervision of the child’s pediatrician, keeping infants upright for a half hour after feeds, can let gravity be your friend and reduce reflux episodes. And in older Children and adolescents, the first and most important thing to do is to do dietary modifications like avoiding trigger foods, caffeine, chocolate, spicy foods and acidic beverages like pop.

Large fatty meals should also be avoided, especially in the two to three hours before bedtime. You should encourage older children to avoid lying down immediately after meals, and in some cases, elevating the head of the bed by 30 degrees can reduce night time symptoms. If you feel that you do need to do pharmacologic management, The most effective drugs are proton pump inhibitors like omeprazole and lansoprazole.

They can reduce gastric acid production and help heal the esophagus. A 4 8 week trial of PPIs is recommended for children with moderate to severe GERD, especially those with esophagitis. So you could start that in the emergency department if the symptoms are severe enough, provided that they have primary doctor follow ups.

You may need to wean these PPIs off if symptoms improve because long term use could be associated with risks such as malabsorption of calcium and magnesium or maybe even an increased risk of GI infections such as C. difficile. H2 blockers or H2 formatidine can be used as an alternative to PPIs in milder cases of GERD.

They start working faster. Again, the PPI’s take three to four days to start working. H2’s work immediately. They do reduce acid production to a lesser degree. In older children and adolescents, antacids can provide symptomatic relief of heartburn, but they don’t address underlying acid production. And then there’s things for infants like myelocon and gas drops.

And if you want to try them, go ahead, but they ain’t gonna stop the physiologic process of reflux. And I’ll touch on surgical management briefly before moving on to part two. In rare, severe cases where GERD is refractory to medical management, surgical interventions like fundoplication may be considered, especially in kids with neurologic impairments or life threatening aspiration.

All right, let us shift gears to gastritis. So gastritis is inflammation of the gastric mucosa and can result from infections such as H. pylori, H. pylori, medication overuse, particularly NSAIDs or other stressors. Children with gastritis often present with epigastric pain, nausea, and vomiting, particularly after meals.

And yes, these symptoms do overlap with gastroesophageal reflux and gastroesophageal reflux disease, but they are separate entities. Now there’s a big caveat here. I know that I’m using the word gastritis. And it’s even in the title of the episode. But technically, this diagnosis is only made after endoscopy and biopsy.

Trust me, I asked a gastroenterologist about this directly. So therefore, if you’re seeing this kid for the first time and there’s no established diagnosis, it’s more accurate, especially when an endoscopy has not yet happened, to diagnose them and label that diagnosis based on their symptoms, such as abdominal pain, nausea, early satiety, endoscopy.

Or, you can label it as dyspepsia, also known as indigestion. This refers to a condition characterized by discomfort or pain in the upper abdomen. Typically manifests as fullness, bloating, nausea, or burning in the stomach, especially after eating. And as noted throughout this episode, dyspepsia can be caused by several factors that overlap with GERD and even a kid who gets an ultimate diagnosis of gastritis, like overeating, spicy or fatty food, stress, or underlying conditions.

And technically, the most common cause is functional dyspepsia. Now, I know that’s a lot to digest, and you might be starting to churn, but let’s talk now about the relationship between eating and pain. A key feature of gastritis is the timing of pain. Pain in gastritis typically occurs shortly after eating, as food stimulates gastric acid production, which can irritate the inflamed stomach lining.

You want to ask detailed questions about the timing in pain in relation to meals. Again, that pain is mostly shortly after eating. The foods that cause more symptoms are the ones you’d expect. Spicy, fatty, acidic, or caffeinated items. A detailed dietary history is one of the first steps to making the diagnosis.

You also want to take a detailed history of medication use, especially NSAIDs. Chronic NSAID use for often comorbid symptoms such as headache can damage the gastric mucosa leading to gastritis. The red flag symptoms, as expected, are similar to GERD. Look for signs of GI bleeding, such as hematemesis or melanoma.

or somebody who appears pale, dehydrated, in severe pain, or worse, even in shock. So if you think a kid has dyspepsia, or maybe even eventually gastritis, it’s the history and physical alone which will make the diagnosis. Now a GI cocktail, which is antacid plus viscous lidocaine, could modify the patient’s current symptoms and help sort of convince them where the pain is coming from.

It has been called into question recently by the adult literature. And probably with good cause, as In an older adult with GI symptoms, you should be worried about myocardial infarction or other causes and if you alleviate or mask the pain then you may be moving yourself away from a more important diagnosis.

There also is the worry about rapid systemic absorption of the lidocaine. In general, I’ve not found them to be incredibly helpful in children, but if you’re not worried about other causes, and you need to modify the kid’s pain to sort of help them convince that it’s their stomach and esophagus as a source of the pain, then it could be worth trying.

I mentioned again that the diagnosis is based on history and physical. If they’ve been dealing with chronic symptoms, And there’s an abrupt worsening, or again, the child has red flag symptoms, then you probably do want to get some labs, such as the CBC, check the HNH, lipase, renal panel, liver profile, and ESR and CRP.

Like a kid with normocytic anemia and an ESR of 140, that ain’t gastritis. I’d be worried about inflammatory bowel disease, for instance. Plain films are generally not helpful unless you suspect obstruction or foreign body ingestion. And then, only a radiopaque foreign body. A CT scan can show some signs of liver disease or pancreatitis, but the HNP followed by targeted labs is a more safe and judicious approach.

Now, ultrasound is useful if you suspect gallbladder disease, but the patient needs to be NPO for six to eight hours before the procedure. This will that the gallbladder is distended, which improves the accuracy of the ultrasound in visualizing gallstones or sludge or other abnormalities. So, a kid with mild symptoms, and you want to rule out gallbladder disease?

Like, you can totally order this as an outpatient. You don’t need to make them wait in the ED for six to eight hours. Have a detailed discussion with the patient and family and do shared decision making in terms of interventions, the GI does, and again, these will be the gold standards. Endoscopy, upper GI and endoscopy is the gold standard for diagnosing gastritis.

You see direct visualization of the gastric mucosa and you take biopsies, which can rule out helicobacter pylori or eosinophilic esophagitis or eosinophilic gastritis. Non invasive H. pylori testing, like stool antigens or a urea breath test, can be helpful for diagnosing H. pylori, especially in cases of chronic or recurrent symptoms.

But, being on a PPI can make these tests less accurate. So really, scope is still the way to go. In the end, the most common diagnoses are All of these kids with gastritis like symptoms are going to end up is functional dyspepsia. This is chronic or recurrent pain and discomfort centered in the left upper abdomen without any identifiable structural or biochemical abnormalities upon medical examination.

Gastritis is, once again, inflammation of the stomach lining, which is only truly identified with endoscopy and biopsy. And yes, I called this episode gastroesophageal reflux and gastritis because it’s a colloquial term, but it’s important to make the distinction that ultimately these kids should be labeled as their symptom or dyspepsia in the ED and then followed up with their primary doctor or GI if necessary.

And many of us will ask about H. pylori. And it’s more common in adults, but it’s actually pretty rare in kids. So unless someone living in the home with a kid has an active infection is being treated. it’s probably not going to be H. pylori in the kid. And still, GI would recommend that we don’t start empiric antibiotic treatment in the emergency department anyway, because it’s best to diagnose that with a scope.

So in the odd, rare scenario where you have a family member being treated for H. pylori and a kid with symptoms, I wouldn’t actually start them on a PPI and instead refer to GI for definitive diagnosis. Other things on the differential include peptic ulcer disease, Again, that’s something that really you’ll pick up on endoscopy, but a kid looks like they’re bleeding out, I’d be worried about it, and they are coming in.

It’s also worth mentioning that gastroparesis and delayed gastric emptying can also cause symptoms of dyspepsia. So what are some other important causes of abdominal pain that you should differentiate from dyspepsia and gastritis? And by differentiate, I mean both ask the right questions and tell the family that you are not worried about them.

First, pancreatitis. So the pain in pancreatitis is typically more severe, constant, can radiate to the back, unlike the more intermittent pain of gastritis. Patients will have more nausea and vomiting and some systemic signs like fever or tachycardia. Elevated lipase can make the diagnosis, though Ultrasound or CT can show some pancreatic inflammation.

Gallstones can be seen in adolescence. Pain in the right upper quadrant or epigastric pain. It’s often described as colicky, worsened by fatty meals. Ultrasound, as I noted before, is the diagnostic test of choice. And peptic ulcer disease from duodenal ulcers improves with food, but then worsens several hours later.

Whereas gastric ulcers may worsen shortly after eating, similar to gastritis. Endoscopy will make the diagnosis. And then there’s celiac disease. So celiac disease can cause dyspepsia symptoms as well. Celiac disease is a true autoimmune disorder triggered by the ingestion of gluten. It’s a protein found in wheat, barley, and rye.

In individuals with celiac disease, gluten intake leads to an immune mediated inflammatory response that damages the small intestine’s mucosal lining, specifically the villi. It kind of blunts them down. This impairs nutrient absorption and can lead to a variety of GI symptoms. So if somebody says, I’m not eating gluten because I don’t feel like it or because I want better hair.

Maybe I should try that. That’s not celiac disease. Celiac disease is an immune process. So dyspepsia is a symptom that can occur in celiac disease, and so if you take a history that things worsen upon eating gluten, or what happens if the patient has eliminated gluten from their diet, which is something that I’ve seen many families do on their own.

And it’s not within the scope of this episode, but the anti tissue transglutaminase antibodies, TTG, IgA, that’s the most sensitive and specific initial test for celiac. All right, let’s start to talk about now the management of dyspepsia and gastritis. Dietary and lifestyle changes are the first line treatment.

They can significantly improve symptoms. It’s important that we communicate to patients and families how optimistic we are that these can make a difference. So avoid trigger foods, spicy and fatty foods, acidic foods and beverages like citrus fruits and carbonated drinks. Caffeine and chocolate, and even dairy products if you suspect lactose intolerance.

You want to encourage eating of small, frequent meals. That allows you to digest the gastric contents a little more efficiently. Chew your food thoroughly. Don’t swallow things like a snake. That reduces the swallowing of air and aids digestion. And avoid late night eating, especially two to three hours before bedtime.

No bedtime snacks for these kids. Kids should stay well hydrated, adequate water intake, and limit sugary drinks like juice and pop that can irritate the stomach. Gatorade. That’s a big offender that I’ve run up against. Stress management is really important because stress can exacerbate symptoms, deep breathing, meditation, biofeedback, regular physical activity promotes healthy digestion, and avoiding secondhand smoke because tobacco smoke can irritate the stomach lining.

If you want some symptomatic relief, you can use antacids. They neutralize stomach acid and provide quick relief from symptoms. So Tums, or calcium carbonate, you chew it and it neutralizes existing stomach acid. It’s quick, short term relief. If you take way too many of them, you can get constipation or hypercalcemia.

Maalox, which is aluminum hydroxide and magnesium hydroxide, it’s two antacids, and it can balance side effects maybe, I don’t know. That also provides symptomatic relief. It’s a little bit gritty, but you drink it. And there are pediatric formulations. If you take a lot of it, magnesium can cause diarrhea, and aluminum can cause constipation.

I don’t know if those balance out at all. I haven’t tried it. Then you have your H2 receptor antagonists, or H2 blockers. These medications reduce stomach acid production by blocking histamine receptors in the stomach lining cells. So you’ve got things like famotidine or pepsid. Runitidine or Zantac was commonly used, but it’s been withdrawn from the market due to some safety concerns.

Lomatidine will decrease acid secretion, and it’s effective for mild to moderate symptoms, and it starts shortly after taking it, and is generally well tolerated. The next step up are proton pump inhibitors. These are more potent acid suppressants, and they block the enzyme responsible for acid secretion itself, as opposed to just the pump.

You’ve got drugs like Prilosec, which is Omeprazole, Prevacid, which is Lansoprazole, or Nexium, Esomeprazole. They all work fine and there’s some pediatric formulations which you can look up and sometimes insurances will take one and not the other, again, beyond the scope of this episode. These are prescribed for more severe symptoms or when H2 blockers are ineffective.

Follow pediatric weight based dosing and know that it does take three to four days before the suppression starts working. Long term use could affect nutrient absorption of like magnesium or vitamin B12. And being on a PPI as opposed to being on an H2 blocker can, as I mentioned earlier, alter the results of an endoscopy.

So if you think a kid’s going to need a scope, don’t start a PPI. Or you can stop the PPI if they’re going to get a scope within the next one to two weeks. Let me talk about one more medication that I’ve used occasionally. And so it’s sucrophate or carophate. This kind of acts like a protective barrier over the stomach lining that can aid in healing.

It’s kind of like putting, like, spackle over the stomach. It just kind of blocks everything up and tamps down the symptoms. It’s a viscous, adhesive substance, and it can adhere to ulcer sites as well. This is really short term treatment for more severe pain. You would administer this on an empty stomach, usually an hour before meals, or when a child is having severe symptoms not around the time of meals.

If you use a lot of it, it can cause constipation or interfere with the absorption of other daily medications. So fortunately, most of the kids with dyspepsia, or kids that you think will ultimately be diagnosed with gastritis, will be able to be discharged home. So first and foremost, recommend dietary and lifestyle modifications.

For You could start a daily proton pump inhibitor and explain to the family that it takes three to four days before it starts working. If they want more immediate relief or the symptoms are mild, you consider using an H2 blocker instead. For mild intermittent symptoms, consider adding Tums if they like to chew a tablet or Maalox if they’d rather drink a liquid.

And for a step up to more severe symptoms, suggest the use of sucrophate and intermittently and judiciously. You should have the child follow up with their primary care doctor often after about 10 to 14 days on the acid blocking regimen that you prescribed. If they’re doing better and they’ve made diet and lifestyle modifications, the primary doctor at that point can discuss how long to maintain therapy and develop an exit strategy.

A trial of four to eight weeks would be reasonable. But if they’re not improving, And that could be a cause for referral to gastroenterology. It’s important in the ED to not over promise that, oh yeah, you’re going to see GI. Kids can get better on what we recommend. You should trust that. So don’t immediately refer to GI unless you’re worried about a bleeding ulcer or another diagnosis like inflammatory bowel disease or celiac or eosinophilic esophagitis because every time they swallow chicken and steak it gets stuck in their esophagus.

Remember, our community pediatricians, family medicine doctors, are brilliant. They can handle this problem. But if a child has those red flags, or if you’re concerned they need immediate intervention, by all means, get them to a pediatric center for admission and GI evaluation imminently. And again, I will say it yet one more time, because most of these kids have not yet had endoscopy with biopsy, I would not recommend using the diagnosis gastritis.

And I did put it in the title, but it’s what most people call it, and perhaps this was a bridge to education. Use symptoms as your diagnosis. Abdominal pain, nausea, early satiety, or probably most appropriately, call it dyspepsia. These kids are having indigestion. Don’t minimize it. It is having a significant impact on their daily lives.

They kept whining about it. They came to the ED. And it is tempting to call it gastritis because it seems more significant or impactful, but Avoiding making a presumptive diagnosis does not minimize the symptoms the child is having. Instead, use this as a teachable moment and link your interventions with your expected symptom improvement.

Alright, so we know that gastroesophageal reflux and gastritis are linked. really dyspepsia, are common yet distinct causes of GI symptoms in pediatric patients. Recognizing the key features of these conditions and differentiating them from other causes of epigastric pain, such as pancreatitis, gallstones, and ulcers, is crucial in providing appropriate management.

Thank you for listening to this episode. I hope you were able to pick up some new pearls that you can take back to your next clinical shift. If you have any feedback on this episode or would like to suggest content for a future episode, send it my way. I’ll take an email, a comment on the blog, a direct message on X or another social media platform.

And my kids will remind me to say, like, rate, and review. That’s so more people can find the show and continue to learn. For PEM Currents, the Pediatric Emergency Medicine Podcast, this has been Brad Sobolewski. See you next time.

This episode of PEM Currents discusses ECPR (Extracorporeal Cardiopulmonary Resuscitation), an advanced procedure used in cases of cardiac arrest when traditional CPR fails. ECPR involves using ECMO (Extracorporeal Membrane Oxygenation) to take over heart and lung functions, offering a last-resort option that is becoming more common in large pediatric hospitals. While ECPR shows promise in improving survival rates, particularly in pediatric patients with conditions like congenital heart disease, it is resource-intensive and carries significant risks. Establishing an ECPR program requires robust infrastructure, multidisciplinary teamwork, and extensive training. The episode highlights the importance of understanding eCPR as a critical therapy for both in-hospital and out-of-hospital cardiac arrests.

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References

Gajkowski EF, Herrera G, Hatton L, et al. ELSO guidelines for adult and pediatric extracorporeal membrane oxygenation circuits. ASAIO J. 2022; 68:133–152. 

Stratton, M., & Edmunds, K. (2024). Extracorporeal Cardiopulmonary Resuscitation. Pediatric Emergency Care, 40(8), 618-622. 

ECC Committee, Subcommittees, and Task Forces of the American Heart Association. 2005 American Heart Association guidelines for cardiopulmonary resuscitation and emergency cardiovascular care. Circulation. 2005;112(suppl):IV1–IV203. 

Yannopoulos D, Kalra R, Kosmopoulos M, et al. Rationale and methods of the advanced R2Eperfusion STrategies for refractory cardiac ARREST (ARREST) trial. Am Heart J. 2020;229:29–39. 

Bartos JA, Yannopoulos D. Starting an extracorporeal cardiopulmonary resuscitation program: success is in the details. Resuscitation. 2023; 187:109792.

Syphilis has gone by many nicknames over the years including “The Great Pretender” and “The Great Imitator.” Emily Labudde, MD, a Pediatric Emergency Medicine fellow at Children’s Healthcare of Atlanta and recent pediatric residency graduate from Cincinnati Children’s discusses the various manifestations of this sexually transmitted infection, and how we can’t miss this very treatable, but sneaky malady.

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References

Emily J. Labudde, Jane Lee; A Review of Syphilis Infection in Pediatric Patients. Pediatr Rev. July 2024; 45 (7): 373–380. https://doi.org/10.1542/pir.2023-006309

Centers for Disease Control and Prevention. “Sexually Transmitted Disease Surveillance 2021.” Centers for Disease Control and Prevention, 2021. Available from: https://www.cdc.gov/std/statistics/2021/default.htm.

Centers for Disease Control and Prevention. “Sexually Transmitted Infections Treatment Guidelines 2021.” Centers for Disease Control and Prevention, 2021. Available from: https://www.cdc.gov/std/treatment-guidelines/syphilis.htm.

Centers for Disease Control and Prevention. “Youth Risk Behavior Surveillance System.” Centers for Disease Control and Prevention, 2021. Available from: https://www.cdc.gov/healthyyouth/data/yrbs/index.htm.

Transcript

Note: This transcript was partially completed with the use of the Descript AI

Welcome to PEMCurrents, the Pediatric Emergency Medicine Podcast, as always, I’m your host Brad Sobolewski. Today’s episode is all about the great pretender, syphilis. And let’s face it, it’s not just a disease for Medieval royalty. It’s on the rise in the United States and abroad. So let’s talk about the manifestations and management.

And I’ve got a special guest host. This is Emily Labudde, originally from Detroit, at the time of recording this episode, a third year categorical pediatric resident at Cincinnati Children’s and a soon to be pediatric emergency medicine fellow in Atlanta. I’m going to pass the mic to you, Emily. My name is Emily Labudde, and I’m a third year pediatric resident at Cincinnati Children’s Hospital, and I’ll be starting fellowship in pediatric emergency medicine this summer at Emory University Children’s Healthcare of Atlanta.

Today’s podcast is going to cover acquired syphilis infection. Now, I know what you’re thinking. We don’t see a lot of syphilis in the pediatric ED. That’s what I thought, too, until I saw it, and with syphilis on the rise, it’s likely we’ll start to see more. I recently published a review article on syphilis in Pediatrics in Review, which you should check out.

It is far more detailed and also covers congenital syphilis, which is beyond the scope of this episode. At the end of this podcast, you should be able to appreciate the rising incidence of syphilis in the United States, especially amongst young people, recognize various signs and symptoms associated with different stages of syphilis infection, and identify the appropriate workup for possible syphilis infection, including co infections.

Syphilis is fondly referred to as the great imitator because of its various presentations. Headache? Could be syphilis. Rash? Could be syphilis. Weight loss? Could be syphilis. Epitrochlear lymphadenopathy? That’ll be on your boards. Definitely syphilis. I bet I could argue to have syphilis on every differential in some way, shape, or form.

But there are certain clues that can help guide you toward a more reasonable consideration of syphilis infection. Thank you, Jen. The U. S. is seeing a lot more cases of syphilis over the last decade or so. And, since we know from the CDC’s Youth Risk Behavior Survey that many American teens are having sex with less than optimal condom use and infrequent STI testing, pediatricians everywhere should be thinking more about acquired syphilis infection, not just congenital syphilis.

There are three stages of syphilis infection. Primary syphilis is often missed as it presents as a painless genital ulcer. Secondary syphilis causes systemic symptoms such as fever, anorexia, headache, malaise, lymphadenopathy, arthralgias, and rash, that classic rash on the palms and soles. It’s worth looking at a variety of pictures of this rash, some examples of which we have included in the show notes, as its appearance can vary, especially between different skin colors.

Tertiary syphilis is rare, and you’re almost guaranteed not to see it in a pediatric ED as it develops decades after initial infection. It’s characterized by gummas, which are granulomatous soft tissue tumors seen most often in the liver, but also in the bone, brain, heart, skin, testis, and eyes, and end organ damage, particularly of the central nervous system.

At any stage, patients can develop neurosyphilis, that can present with vision changes, neuropathies, seizures, or altered mental status. You may have heard this once or twice before, but your history and physical exam are so important, especially when looking for an infection like syphilis that can present in many different ways.

A thorough review of systems can help guide your differential and can point towards co infections with other STIs. Brush up on your sexual history taking skills because they’re critical here. This includes all the details like who has what parts and where they put them. Teens are awkward and they get super nervous when you kick out mom and dad and ask them what they’ve been doing on the weekends, but you won’t fully understand your patient’s risks without asking these questions.

It’s good preparation prior to the physical exam, which, you guessed it, should be thorough. We’re talking full body skin exam, neuro exam, genital exam, and all of the lymph nodes. Now, it would be really nice if our patients came in saying, Hey, my partner has syphilis, please test me for syphilis. And, like we talked about, syphilis can look like a lot of different things, so it’s important to keep your differential broad.

Syphilis testing can be divided into two types, treponemal and non treponemal. You’ll want to start with a non treponemal test, such as an RPR or VDRL. The RPR or Rapid Plasma Reagent, which has now largely replaced the earlier VDRL or Venereal Disease Research. Laboratory Test is a non-specific serological test for syphilis that uses CARDIOLIPIN as antigen.

These are highly sensitive tests. But watch out for false positives. The RPR is also useful for post treatment monitoring. Trepanemal tests, such as the FTA ABS, are used as confirmation in the setting of a positive non trepanemal test. Patients with neurologic complaints should undergo CSF testing as well.

And, of course, where there’s one, there’s probably more. Use the information from your very thorough sexual history to test your patient for any other STIs they are at risk for. Patients with primary or secondary syphilis can be treated with a single dose of benzathine penicillin G or a 14 day course of doxycycline if your patient has a penicillin allergy.

For any patient with tertiary or neurosyphilis or a pregnant patient with any stage of infection, 10 to 14 days of penicillin G is the only option, even in penicillin allergic patients. So, if a patient has a severe penicillin allergy, have rescue medications available. Watch out for that Gerrish Herxheimer reaction, I know you haven’t heard those words since medical school, which can cause fever, headache, myalgias, or nausea and vomiting in the first 24 hours after treatment.

Last, but not least, remember that in some cases you’re telling your patient about their disease and possibly some dishonesty from their partner. Be sure to counsel them on sharing their results with all of their sexual partners, as well as your responsibility to inform the local health department for disease tracking purposes and the option for third party partner notification.

A few brief points on congenital syphilis, which is covered more in depth in the review article. We most often see this in infants born to mothers with poor prenatal care, as our OB colleagues do a great job screening for syphilis multiple times during pregnancy. Most infants with congenital syphilis are asymptomatic at birth, with appearance of hepatomegaly, jaundice, copious rhinorrhea, lymphadenopathy, and a similar maculopapular rash on their hands and feet that develop later in infancy.

Think about congenital syphilis in your little ones presenting like biliary atresia, who have a normal gallbladder treon ultrasound. Late congenital syphilis in children older than two can present with more severe features that are frequently tested on boards. Things like gummas, facial dysmorphias like saddle nose deformity and frontal bossing, sabershins, Hutchinson teeth, developmental delay, and hearing and vision concerns.

You’ll want to check a non trepanemal test in these kids, as maternal trepanemal antibodies can persist for over 15 months. Patients with congenital syphilis also receive treatment with penicillin G, 50, 000 units per kilo, frequency determined by their age. Thanks so much for listening, now get out there and practice your sexual histories.

Emily, thank you very much. Hopefully you all found this information helpful and we’ll be able to pick syphilis out of the lineup the next time you encounter it in the emergency department. If you want to learn how to produce a podcast episode, reach out to me, just like Emily did, and we will go through the entire process.

If you’ve got ideas for topics, send them my way. Any feedback that you have is greatly appreciated. Send me an email, leave a comment on the blog, a review on your favorite podcast site, or even a message through social media. And as my 12 year old would remind me to say, subscribe and share. For Pam Currens, the Pediatric Emergency Medicine Podcast, this has been Brad Sobolewski.

See you next time.

Cervical Spine Injuries are fortunately rare in children. this episode is all about learning when to suspect them, how to immobilize the C-spine properly, and which imaging test to choose. It was inspired by a hot-off-the-presses publication from the Pediatric Emergency Care Applied Research Network (PECARN) focused on clinical decision rules for cervical spine imaging in children.

Check out the paper by Leonard et al. entitled “PECARN prediction rule for cervical spine imaging of children presenting to the emergency department with blunt trauma: a multicentre prospective observational study” at Lancet Child & Adolescent Health from Leonard et al here!

Maybe there’s a funky music video that will teach you how to clear the C-spine?

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References

Leonard, J. C., Harding, M., Cook, L. J., Leonard, J. R., Adelgais, K. M., Ahmad, F. A., Browne, L. R., Burger, R. K., Chaudhari, P., Corwin, D. J., Glomb, N. W., Lee, L. K., Owusu-Ansah, S., Riney, L. C., Rogers, A. J., Rubalcava, D. M., Sapien, R. E., Szadkowski, M. A., Tzimenatos, L., Ward, C. E., Yen, K., Kuppermann, N. (2024). PECARN prediction rule for cervical spine imaging of children presenting to the emergency department with blunt trauma: a multicentre prospective observational study. Lancet Child & Adolescent Health. https://doi.org/10.1016/S2352-4642(24)00104-4.

Sasser SM, Hunt RC, Faul M, Sugerman D, Pearson WS, Dulski T, Wald MM, Jurkovich GJ, Newgard CD, Lerner EB; Centers for Disease Control and Prevention (CDC). Guidelines for field triage of injured patients: recommendations of the National Expert Panel on Field Triage, 2011. MMWR Recomm Rep. 2012 Jan 13;61(RR-1):1-20. PMID: 22237112.

Leonard JR, Jaffe DM, Kuppermann N, Olsen CS, Leonard JC; Pediatric Emergency Care Applied Research Network (PECARN) Cervical Spine Study Group. Cervical spine injury patterns in children. Pediatrics. 2014 May;133(5):e1179-88. doi: 10.1542/peds.2013-3505. PMID: 24777222; PMCID: PMC9923608.

Baker C, Kadish H, Schunk JE. Evaluation of pediatric cervical spine injuries. Am J Emerg Med. 1999 May;17(3):230-4. doi: 10.1016/s0735-6757(99)90111-0. PMID: 10337876.

Leonard JC, Browne LR, Ahmad FA, Schwartz H, Wallendorf M, Leonard JR, Lerner EB, Kuppermann N. Cervical Spine Injury Risk Factors in Children With Blunt Trauma. Pediatrics. 2019 Jul;144(1):e20183221. doi: 10.1542/peds.2018-3221. PMID: 31221898; PMCID: PMC6615532.

Leonard JC, Jaffe DM, Olsen CS, Kuppermann N. Age-related differences in factors associated with cervical spine injuries in children. Acad Emerg Med. 2015 Apr;22(4):441-6. doi: 10.1111/acem.12637. Epub 2015 Mar 16. PMID: 25779934.

Leonard JC, Kuppermann N, Olsen C, Babcock-Cimpello L, Brown K, Mahajan P, Adelgais KM, Anders J, Borgialli D, Donoghue A, Hoyle JD Jr, Kim E, Leonard JR, Lillis KA, Nigrovic LE, Powell EC, Rebella G, Reeves SD, Rogers AJ, Stankovic C, Teshome G, Jaffe DM; Pediatric Emergency Care Applied Research Network. Factors associated with cervical spine injury in children after blunt trauma. Ann Emerg Med. 2011 Aug;58(2):145-55. doi: 10.1016/j.annemergmed.2010.08.038. Epub 2010 Oct 29. PMID: 21035905.

Transcript

Note: This transcript was partially completed with the use of the Descript AI

Welcome to PEM Currents, the Pediatric Emergency Medicine Podcast. As always, I’m your host Brad Sobolewski, and this episode is all about cervical spine injuries in children. Now, fortunately, cervical spine injuries in kids are rare. They only happen in about 1- 2 percent of pediatric blunt trauma injuries.

But, In children with cervical spine injuries, at least one in five have permanent neurologic deficits, and serious cervical spine injuries, there’s a 7 percent mortality rate. So severe mechanisms are the scenarios where you are most likely to see a C spine injury in kids. So these are motor vehicle collisions with a patient ejected from the car, motor vehicle collision with death of another occupant, and Intrusion into the patient’s passenger compartment of greater than 12 inches at the roof and or greater than 18 inches at any site. So you got to ask the prehospital personnel about the injury and the crash scene fall of a distance greater than 10 feet or two to three times the child’s height diving into a body of water and an axial load. So force applied to the top of the head and acceleration deceleration Injury of the head.

So you hit your head on a dashboard during a head on collision. A clotheslining force. So that’s caused by a rope, a cable, or another object exerting traction on or striking the neck while the body is in forward motion. And certain sports do have a higher association with cervical spine injuries like football, hockey, wrestling, bicycling, trampoline use or riding ATVs. Infants can get a cervical spine injury during breech delivery or, unfortunately, during non accidental trauma as well. Axial injuries, occiput to C2, are much more commonly seen in children under the age of eight – it’s three quarters of all cervical spine injuries. These are most often due to motor vehicle collisions and falls.

Kids this age are more susceptible because of their big lollipop heads. You know, they have a giant head size related to their body size and they have loose joints and ligaments overall. Their C spine fulcrum is higher at birth, it’s at C2 to C3, as opposed to C5 to C6, which is the usual position in older children and adolescents just through the process of normal growth.

The most common injuries seen in these younger patients are growth plate fractures and ligamentous injuries. It’s particularly difficult to diagnose cervical spine injuries in kids under three because they can’t give you an accurate history and cooperate with the exam. Older children, so older than eight, so middle schoolers and up, have a higher likelihood of injuries in the C3 to C7 range.

This makes up about half of the injuries. And these happen during motor vehicle collisions and sports. You more often will see vertebral body and arch fractures as opposed to the growth plate fractures and ligamentous injuries in the younger children. And overall, C spine injury can occur through a lot of different mechanisms including flexion, extension, vertical compression, rotation, or combination of all of the above.

And though we’re talking about injuries to the vertebra, the cervical spine, spinal cord injuries themselves happen either due to direct compression, or disruption of the cord itself by a fracture, fragment, or a sublux vertebra. Let’s go ahead and pivot to initial management. And we need to suspect cervical spine injuries in any patient with multisystem blunt force trauma.

You want to limit spine motion during your primary survey, the ABCs, or the rapid cardiopulmonary assessment. Someone can hold c spine, and we’ll talk about more about maintaining and clearing the c spine in a little bit. You’ll do jaw thrust alone as an airway maneuver. No head tilt. Orotracheal intubation with video laryngoscopy is ideal.

C spine injuries themselves can impact airway maintenance and or patency. So if you have an unstable injury above C3, you can actually have respiratory paralysis. A lower cervical injury could impact the phrenic nerve. The cervical spinal column injury itself may be associated with airway obstruction from retropharyngeal hemorrhage, edema, or maxillofacial trauma.

You should also consider the possibility of quote unquote spinal shock. This is due to the loss of sympathetic output and vasodilatation. So you could worry about this in a bradycardic and hypotensive patient, but in multisystem trauma hypotension is more likely hemorrhage. than it is spinal shock.

Resuscitate with volume and blood. When evaluating the cervical spine in particular, if you can get details on the mechanism, that’s fantastic. You’ve got some specific injury patterns that you should be on the lookout for. So a patient that has hyperflexion can have a vertebral body wedge fracture and disruption of the posterior elements.

A hyperextension Extension injury will compress the posterior elements and disrupt the anterior longitudinal ligament. This is the hangman’s fracture, the posterior neural arch of C1 or the pedicles of C2. Axial load can cause burst fracture, so someone that dives into a pool. A rotational injury will disrupt the facets.

This is more common in combination with an extension or flexion injury, not just rotation alone. And then there’s the specific Atlanto Axial Rotary Subluxation Pattern. It’s often minor trauma in younger children, where C1 and C2 essentially get stuck on each other in a rotary position, and the kid can’t turn their neck.

Children will have some localized cervical pain, muscle spasm, decreased neck range of motion. They may or may not have neurological symptoms, even if they’ve resolved. These can include paresthesias, numbness, or weakness. The distribution of these neurologic symptoms is really variable, and it can range from involvement of single dermatomes to dramatic neurologic deficits including quadriplegia.

The ability to walk does not completely exclude a C spine injury. And interestingly, even children with no symptoms can have a cervical spine injury. There is a retrospective review of children just before the turn of the millennium that found that even 10 percent of that population with cervical spine injury were initially asymptomatic.

Finally, there are some children that you need to be aware are more predisposed to cervical spine injury than others. And this includes children with Down syndrome, clipple feel, osteogenesis imperfecta, marfans, Ehlers Danlos, chronic steroid use, rickets, and more. When it comes to physical examination of a child with a suspected cervical spine injury, remember, immobilize the c spine, either by hand, so another team member holding inline c spine, or by placing a collar, like an Aspen or Miami.

The ABCs are, as always, incredibly important. An axial injury, occiput to C2, causes abrupt cessation of respiration, so that patient will be apneic. A patient who is hypoventilating may have injuries of the spinal cord at the level of diaphragmatic control, so C3, C4, C5. And hypotension, bradycardia, or temperature instability can result from hemorrhagic and or spinal shock.

When examining the neck, you want to maintain in line stabilization. You have to palpate, but don’t press too hard on the spinous processes for local tenderness, muscle spasm, or obvious deformity. Either start at C7 and work your way up, or find C1 and work your way down. Be deliberate about touching each cervical vertebrae.

Tell younger children to use their words. Yes if it hurts, no if it doesn’t. Don’t shake their head. They always do this. A child with midline cervical tenderness. is more likely to have a cervical spine injury than a child with paraspinous muscular tenderness or spasm. Infants and toddlers who can’t cooperate can actually be cleared without imaging after minor trauma if they have a normal neurologic examination that includes mental status and GCS 15 and no other life threatening injuries.

It’s really hard to know if an 18 month old has C spine pain or not. In my experience, doing a neurologic exam is really difficult in the resuscitation area, especially when a patient is supine and has an immobilized c spine. The overall Glasgow Coma Scale and evaluation of tone, strength, sensation, and reflexes constitutes a complete neurologic exam.

50 percent of all children with cervical spine injuries will have some sort of neurological deficit. And yes, doing a neuro exam is hard, and it takes practice. Part of that is learning ways to get patients to participate when they are uncomfortable or scared. And subtle findings are fortunately most common, but harder to elicit.

An isolated sensory deficit is the most common neurological finding in cervical spine injury. Ipsilateral posterior spinal column and contralateral anterior column are tested via light touch. The anterolateral spinal column is tested with pinprick or pain. The ipsilateral posterior spinal column is tested with position sense, so moving that toe, and dysesthesia will localize to the central cord.

And this is a podcast, so I can’t make you conjure up a table in your head, but I think it is important to remember where some deficits will come from if you have injuries at particular levels. And so if you have an injury at C2 to C3, you may just see apnea. C3 and C4 control the diaphragm, so you want to make sure the patient is spontaneously breathing.

C5 is flexion of the biceps. with the palm up, or supinated. C6 is extension of the wrist. C7 is extension of the elbow. L2 through L4 are extension of the knee. L5 is dorsiflexion of the great toe. And S3 and 4 is rectal tone. So absence of rectal tone is a poor prognostic sign, but it’s also not sensitive for cervical spine injury.

In a child with a GCS of 15 with no active neurological complaints, I would argue that a digital rectal exam is pretty invasive, and just squeezing the buttocks together is probably a good proxy. A child with altered mental status, or decreased responsiveness, or high index of suspicion for cervical spine injury should get a digital rectal examination.

And yes, on board exams! You have these spinal cord injury syndromes. They show up again and again and again. I’ve actually rarely seen them in pediatric practice, fortunately. But for completeness, here they are. Anterior cord syndromes are from hyperflexion, and you’ll see paralysis and loss of pain sensation without loss of light touch or proprioception.

Central cord syndromes are from hyperextension, and you’ll see weakness that is greater in the upper as opposed to the lower extremities, and transient burning sensation of the hands and fingers. Brown Sequard syndrome, which is cord hemisection, will lead to ipsilateral paralysis, loss of proprioception, and loss of light touch, and a contralateral loss of pain and temperature sensation.

And then Horner syndrome is disruption of the sympathetic chain. So you’ll see ipsilateral ptosis, meiosis, and anhydrosis. Okay, so let’s say the ABCs are normal, GCS is 15, and there are no focal neurologic findings. How do we attempt to clear the C spine? So for this, you’re gonna need another team member.

So have the patient lie supine. Have your assistant hold inline C-spine mobilization. They’re gonna be standing above the patient’s head, and then you’ll remove the anterior front portion of the collar. Reach behind their neck, inside the back portion of the collar, and feel very intentionally. See one all the way down to C seven.

Or the reverse, C7 all the way up to C1, asking the patient at each one if there is pain. And again, tell them to use their words, yes or no, and not nod or shake their head. And they’ll still mess this up. At each cervical vertebrae, feel for any swelling or step off. So one that feels kind of more in than the rest.

If there is pain at any cervical vertebrae, replace the collar. And then you’ll move on to imaging, which we’re going to talk about in just a moment. If there is absolutely no pain in C1 through C7, You can have your teammate release inline c spinal mobilization and then ask the patient to actively flex, extend, and rotate to the left and right, all at 45 degrees.

If they have no pain in the midline with any of these movements, then the cervical spine is clinically cleared. But if they have pain or decreased range of motion in any direction, replace the collar and move on to imaging. No matter where you work, you should apply clinical decision rules with an imaging algorithm for anybody with suspected cervical spine injuries.

Now in grown ups, you’re probably familiar with the Canadian C spine rule. It’s a highly sensitive rule that is designed to prevent missing cervical spine injuries while limiting the amount of unnecessary radiologic examinations. This Canadian C spine rule does not apply to children under 16 years of age.

In many settings in adults, plain radiographs have actually been abandoned in favor of CT scans. And a negative CT scan, if you rule in via the Canadian C spine rule, is generally sufficient to clear the majority of C spine injuries and allows for collar removal. In children, though, we prioritize limiting radiation risk.

And so it’s recommended, based on recent evidence from the Pediatric Emergency Care Applied Research Network, PCARD, that we have a three tiered decision rule. So the highest risk patients, these patients have a risk of cervical spine injury of about 12%, are going to recommend immediate medical attention.

CAT scan. These patients will have altered mental status, A GCS of three to eight or unresponsive on the AVPU alert, voice pain, unresponsive, mnemonic. Highest risk patients also include those with any abnormality of the airway, breathing, or circulation. and somebody with a focal neurological deficit. And then there’s an intermediate risk group that has a just under 4%, specifically a 3.

6 percent chance of a cervical spine injury. And these are patients that it is recommended to get a plain x ray. These are patients with posterior neck pain, altered mental status, but a GCS of greater than eight. Or patients with a substantial head or torso injury and substantial means that they’re going to require an intervention or observation in the hospital.

And then there’s a low risk group with a risk of cervical spine injuries of 0. 2 percent or less. And these patients generally don’t need any imaging. And so this is, uh, Assuring through meticulous investigation that none of the following risk factors are present. So neck pain or midline posterior neck tenderness, decreased range of motion or pain with range of motion on flexion, extension, or rotation to the left and right, torticollis, altered mental status so a GCS of 14 or less, any focal neurologic finding and remember sensory deficits are the most common in cervical spine injuries, Any substantial coexisting injuries, so especially torso injuries or child abuse injuries.

Any relevant predisposing condition like Down syndrome. And high risk mechanisms. Diving, hanging, an axial load force, a clothes lining force, or a motor vehicle collision with significant intrusion, ejection from the vehicle, or a death in the vehicle. So again, if the child is negative for all of those, you don’t have to place a collar.

And you don’t need any imaging, and that includes an x ray or a CT scan. The goal of using this high intermediate, low tiered approach is to cut the rate of CAT scans in children by greater than 50%. And so the Pediatric Emergency Care Applied Research Network is actively working on that, initially in an ED setting, with future work in the pre hospital setting.

And so hopefully that helps you understand when to get imaging. But what about the actual imaging choices themselves? Plain x rays are the initial choice in children with normal mental status, but cervical spine tenderness. They’ve got adequate sensitivity to exclude unstable c spine injuries. There are two view x ray series of the neck, that’s an AP and a lateral, or a three view series, cross table lateral, AP, and when obtainable, then open mouthed odontoid.

Multiple views, as you’d expect, are more sensitive, like 90%, as opposed to a single view, which is only 79%. Some children are chunky, and it’s hard to see all seven cervical vertebrae, but you do need to see that for a complete set of film. Some of you might work at a place where they get the swimmer’s view, where they grab both patients hands or wrists, and then pull down to try to get the shoulders out of the way to show C7.

If you have a high suspicion for cervical spine injury, don’t do this. Flexion extension views have fallen out of favor in my practice environment because CT is readily available. The FlexX views are still used in some situations, and they could show some ligamentous disruption. This is where the patient actively flexes and extends with X rays taken.

Never do passive flexion of a suspected C spine injury. CT or CAT scans, by default, get axial images. computed axial tomography. Then the computer is going to do fancy sagittal, coronal, and 3D reconstruction, which is totally cool. CT scans are indicated in any child with altered mental status, a GCS of 3 to 8, or unresponsive on the AFPU.

Also, in children with an abnormal airway breathing and or circulation. and orifocal neurologic deficit. The risk of radiation is the primary concern here, and radiology departments should follow the ALERA, or as low as reasonably acceptable, principle. A C spine CT delivers substantially more radiation to the skin, thyroid, and spinal cord, up to 10 50 percent more.

Children younger than 5 years of age are more prone to radiation induced malignancies due to the increased radio sensitivity of certain organs and a longer latency or life period to develop a cancer. Calculating the lifetime risk of getting cancer from CT scans is hard to do, which is great because we’re not just like scanning people for no reason and seeing if they get cancer later.

The best current estimate is that the estimated lifetime cancer mortality risk attributable to the radiation exposure from a CT for a one year old is approximately 0. 07 to 0. 18%. So not zero, but pretty low. The risk of radiation exposure exceeds the benefit of CT imaging in the majority of children evaluated for C spine injury.

So most of them don’t have a very low GCS, or abnormal ABCs, or focal neurologic deficits. That’s why PCARN is doing this work. Many adult centers will readily get CT scans in adults with suspected c spine injuries. These are just not necessary in the majority of children. Either nothing or plain x rays are sufficient.

And what about MRI? It’s becoming increasingly available. In anybody with an abnormal neurologic examination, or when imaging of the spinal cord or other soft tissues is paramount, MRI can be very helpful. For It is superior to CT for visualizing soft tissues and identifying intervertebral disc herniation, ligamentous injuries, and spinal cord edema, as well as hemorrhage, compression, and transection type injuries.

MRI is actually less sensitive than CT for detection of fractures of the posterior elements of the c spine and injuries to the cranial cervical junction. So it’s not perfect. Even fast protocols for MRIs are tough to get in children under the age of 6 years, they require sedation. And spinal cord injury without radiographic abnormality, C.

Wura, was defined way back in 1982, and I was 4 or 5 years old when this happened. And this was objective signs of myelopathy as a result of trauma in the absence of findings on plane radiographs, flexion extension radiographs, and cervical CT. CWRA is kind of a moot point when you have an imaging modality that uses fancy magnets to jiggle water molecules and take a cool picture.

You can demonstrate injury to the spinal cord and spinal ligaments. And so in anybody with a localizable neurologic sign or symptom, I’m not saying you have to get it in the ED because that’s just not practical, but these patients will, upon admission or shortly after their initial assessment, need an MRI at some point.

And though this episode is focused on cervical spine injuries, it goes without saying that if somebody has thoracic or lumbar spine pain, they should get plain imaging of that, or if they have substantial multisystem trauma and you’re getting a CT scan of the chest or abdomen, that is obviously going to include those bones as well.

Now moving on to disposition. Any patient with a cervical spine injury or a neurologic deficit They’re getting admitted to the hospital. And this obviously includes patients who need surgery, like unstable fractures and those sort of things. That’s a spine or neurosurgery operation. Patients with stable fractures, so an isolated spinous process, or transverse process fractured, identified by CT, will have a rigid cervical collar applied, so an aspen or vista, With trauma and spine follow up within a week.

Kids wear this collar 24 7. So again, you’ve got an isolated, stable fracture, no displacement, no neurologic symptoms, no other injuries, that kid could go home and they’re going to wear that collar 24 7. You have to teach them how to take care of it. If you have a negative x ray, but persistent midline c spine pain, we also recommend keeping the kid in the cervical collar and follow up at a trauma or spine center within a week.

Could you get a CT scan in those situations? Yes, but still most pediatric trauma centers will void the ionizing radiation of the CT scan and keep the patient in the collar until trauma follow up. Okay, so let’s wrap up this episode. Fortunately, cervical spine injuries in children are rare. You should learn how to clinically clear a C spine and know that it takes two people to do it correctly.

Practice your neurologic examination in children who are being evaluated for traumatic injuries. Know which mechanisms are more likely to cause C spine injuries. And make sure that you’re using a clinical decision rule with an imaging algorithm for kids with suspected C spine injuries. Highest risk patients should get CT scans.

They have a 1 in 8 chance of a c spine injury, altered mental status, GCS 3 to 8, unresponsive, abnormal ABCs or focal neurologic deficit. Patients that are intermediate risk have a less than 1 in 25 chance of a c spine injury and that’s when we would get a plain x ray. So they have Posterior midline neck pain, altered mental status, but a GCS of greater than 8, or some substantial comorbid head or thoracic injury that requires management or admission to the hospital.

Patients with no findings will have a less than 0. 2 to 0. 3 percent chance of a cervical spine injury and don’t need any imaging at all. So this is a patient with no midline neck pain, no pain on neck range of motion, Normal mental status, normal neurologic exam, no comorbidities, and no high risk mechanisms.

I encourage you all to take a look at the new publication from the PCAR Network in Lancet Child and Adolescent Health. I’ve provided a link in the show notes. This is the state of the art paper on the use of decision rules for cervical spine imaging in children. To learn more about the Pediatric Emergency Care Applied Research Network, or PCARN, check out PCARN.

org. You can also follow them on x at pkarn team. If you’ve got feedback on this episode, send it my way. Email, direct message on x, a comment on the blog, I’d love to hear it. Hopefully you found this information useful and you can take it back to your next shift. Overall, that’s the goal of this podcast.

Encourage your colleagues to listen and subscribe. Hopefully they will find it helpful as well. And please let me know if there are any topics specifically related to trauma and injuries in children that you think that I should cover. For PEMCurrents, the Pediatric Emergency Medicine Podcast, this has been Brad Sobolewski.

See you next time.

Febrile Seizures are among the most common neurological problema in young children, occurring in 1 out of 50 children between the ages of 6 months and 5 years of age. This episode of PEM Currents: The Pediatric Emergency Medicine Podcast is a Question and Answer style exploration of some of the most common learning points in this incredibly important topic.

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References

Xixis KL, Samanta D, Smith T, et al. Febrile Seizure. [Updated 2024 Jan 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK448123/

Feenstra B, Pasternak B, Geller F, et al. Common variants associated with general and MMR vaccine-related febrile seizures. Nat Genet 2014; 46:1274.

Mullan PC, Levasseur KA, Bajaj L, Nypaver M, Chamberlain JM, Thull-Freedman J, Ostrow O, Jain S. Recommendations for Choosing Wisely in Pediatric Emergency Medicine: Five Opportunities to Improve Value. Ann Emerg Med. 2024 Feb 11:S0196-0644(24)00017-9. doi: 10.1016/j.annemergmed.2024.01.007. Epub ahead of print. PMID: 38349290.

Guedj R, Chappuy H, Titomanlio L, De Pontual L, Biscardi S, Nissack-Obiketeki G, Pellegrino B, Charara O, Angoulvant F, Denis J, Levy C, Cohen R, Loschi S, Leger PL, Carbajal R. Do All Children Who Present With a Complex Febrile Seizure Need a Lumbar Puncture? Ann Emerg Med. 2017 Jul;70(1):52-62.e6. doi: 10.1016/j.annemergmed.2016.11.024. Epub 2017 Mar 2. PMID: 28259480.

Shinnar S, Hesdorffer DC, Nordli DR Jr, Pellock JM, O’Dell C, Lewis DV, Frank LM, Moshé SL, Epstein LG, Marmarou A, Bagiella E; FEBSTAT Study Team. Phenomenology of prolonged febrile seizures: results of the FEBSTAT study. Neurology. 2008 Jul 15;71(3):170-6. doi: 10.1212/01.wnl.0000310774.01185.97. Epub 2008 Jun 4. PMID: 18525033.

Murata S, Okasora K, Tanabe T, Ogino M, Yamazaki S, Oba C, Syabana K, Nomura S, Shirasu A, Inoue K, Kashiwagi M, Tamai H. Acetaminophen and Febrile Seizure Recurrences During the Same Fever Episode. Pediatrics. 2018 Nov;142(5):e20181009. doi: 10.1542/peds.2018-1009. Epub 2018 Oct 8. PMID: 30297499.

Transcript

Note: This transcript was partially completed with the use of the Descript AI

Welcome to PEMCurrents, the Pediatric Emergency Medicine Podcast. As always, I’m your host, Brad Sobolewski. This episode is all about febrile seizures, one of the most common neurological problems that you will see in the emergency department in children. And you know what? I’m going to structure this episode like a bit of a question and answer session.

I’ll ask a question, and then I’ll answer it. So the first and perhaps most important question is, What are febrile seizures and how common are they? Well, they are the most common neurologic disorder of infants and young children, and they happen in about 2 to 4 percent of children between the ages of 6 months and 5 years of age.

I’ll tell parents that they happen in about 1 out of 50 kids. It’s associated with fever, but in a child without evidence of intracranial infection. They are not considered a form of epilepsy. They peak between 12 and 18 months of age, the male to female ratio is 1. 6 to 1, and there is a higher prevalence reported in certain regions like Japan’s Mariana Islands.

A febrile seizure, very simply, is a convulsion associated with a temperature greater than 38 degrees Celsius. The child does not have any acute systemic metabolic problems like hypoglycemia or hyponatremia, and you don’t have to test for those. We’ll talk about that later. And they have no history of previous afebrile seizures.

So why do they happen? Well, they’re likely related to a vulnerability of the developing nervous system to the effects of fever. The neurons that generate these seizures aren’t completely myelinated until age 6. And these neurons that are undermyelinated are more hyper excitable by cytokines during fever in these younger children who get sick more often.

And of course, underlying genetic susceptibility plays a role. And so other risk factors include a high fever, A viral infection, certainly particular viruses. A recent immunization. Hmm. Family history of febrile seizures. Prenatal exposure to nicotine. Atopic diseases. And maybe iron deficiency anemia. And so check this out.

Febrile seizures are due to the degree of fever, not the rate of temperature rise, even though we see them as the temperature is increasing rapidly in the early parts of the illness. And this has been known since the 1950s. The majority of children have febrile seizures on the first day of illness. In some cases, it’s actually their first manifestation of illness.

Like, they’re just a little bit congested, and then they’re convulsing. And then they find out that they’re febrile. The degree of fever associated with febrile seizures is variable, and it depends on the kid’s threshold convulsive temperature. So everybody has a little bit of a different set point. But most often, the fever is greater than 39 degrees Celsius, but 25 percent of febrile seizures do occur between the temperatures of 38 and 39.

In a study of just over 100 children, the temperature of febrile seizures was significantly higher than the mean temperature of fevers in children that did not have seizures, so 104 versus 103. 3. The seizure threshold is lower in infants. who have more febrile illnesses. So viral infections are often associated with high fever in kids, you know that.

One of the most classic viruses associated with febrile seizures is HHV 6. So human herpes virus 6. Another common one is influenza, specifically influenza A. So HHV 6 is the cause in one third of all first time febrile seizures in U. S. children under 2 years of age. The mean maximum fever in infants with a primary HHV 6 infection is generally 39. 5 Celsius, so 103 Fahrenheit or higher. So the incidence of febrile seizures associated with primary HHV 6 infection is estimated as high as 36 percent in 12 to 15 month olds. Other common causes include adenovirus RSV, HSV, CMV, HHV 7, and in Asia, influenza A is number one. The type of viral infection is not important in predicting the future recurrence of febrile seizures or whether or not the child will have a complex febrile seizure.

In kids up to two and a half years of age, breastfeeding may be a preventative factor for febrile seizures. Vaccines don’t cause autism, but they might cause febrile seizures. So the risk of febrile seizures is increased after administration of diphtheria, tetanus, toxoid, and whole cell pertussis, along with measles, mumps, rubella, and MMR with varicella vaccines.

The absolute risk is small, and genetic susceptibility likely plays a role in seizures after vaccines. The risk of a future febrile seizure with a subsequent vaccine is generally lower than the risk of the disease that you are vaccinating against. In drug company sponsored studies, the absolute risk of a febrile seizure after an MMRV vaccine is about 3 to 4 febrile seizures for every 100, 000 children receiving the vaccine.

So again, pretty darn low. So how do we categorize febrile seizures? We all know that there’s simple, That may not be the best terms, but that’s what we’ve got. So the focality, in a simple febrile seizure, they are generalized. A complex febrile seizure is focal, so the shaking is limited to one limb or one side of the body.

The duration, the duration for simple febrile seizures is less than 15 minutes, though 10 minutes has been proposed. Complex febrile seizures, on the other hand, are longer than 15 minutes. A simple febrile seizure is limited to a single episode in a 24 hour period, whereas complex febrile seizures, there is a recurrence of more than once in that 24 hour period.

Overall, 80 percent or more febrile seizures are simple, and 20 percent are complex. Ultimately, though, the history that you get may not be reliable. Both the motor movements and characteristics of the seizure, as well as the duration, are really hard to assess in a patient’s home. Obviously, you should ask about immunization status and whether or not the child has any underlying medical or neurologic problems or developmental delay.

And let’s be honest, the term simple sort of undersells how scary this is for families. There’s nothing simple about watching their child have convulsions and looking like they were gonna die. And why does the complex heterogeneity? Think about it this way. Two one minute seizures occurring an hour apart in a well appearing febrile child seem different than focal one sided convulsions or febrile status epilepticus.

More research is needed. So what’s the recurrence risk after febrile seizures? This is an important thing that we talk to families about. So the overall recurrence rate is approximately 30 to 35%. I will tell parents it’s a one in three chance. If you have two or more febrile seizures, you have a 50% chance of subsequent events.

The subsequent seizure is almost always similar to the first. So 95% of initial simple febrile seizures have recurrent, simple febrile seizures. And interestingly, they’re usually about the same length as well. The risk of recurrence is much higher in children under 12 months of age. So, though it’s one third overall, it’s about 50- 65 percent in children under 12 months of age when they have their first febrile suture.

In older children, like preschool and above, the risk is less than 20 percent. In a study published by Berg in Archives of Pediatrics and Adolescent Medicine in 1997, when I was a freshman in college, they looked prospectively at 428 children with a first febrile seizure. They noted that 17 percent had one recurrence, 9 percent had two recurrences, and 6 percent had three or more.

Three quarters of these recurrences were within one year of the initial seizure, and almost all were within two years. They found four factors for increased recurrence risk. Young age, history of febrile seizures in a first degree relative, lower degree of fever while in the emergency department, and brief duration between the onset of fever and the initial seizure.

Kids with all four of those had a 70 percent risk of recurrence, none of them only 20%. Complex features on the initial seizure were not associated with the risk of recurrence. Other factors that have been reported regarding recurrence in the literature include abnormal development before the first febrile seizure, recurrence of seizures within the same illness, children with one recurrence, and of course children who have had an unprovoked seizure after a febrile seizure are more likely to have future febrile seizures.

So what’s the risk of epilepsy following a febrile seizure? Well, 1% One out of a hundred human beings have epilepsy. If you have one simple febrile seizure, your risk of epilepsy is somewhere between 1 and 1. 5 percent. So it really doesn’t go up significantly. In a normal child with a simple febrile seizure, that risk is only slightly above that of the general population.

So they don’t really need a neurologic workup. Complex febrile seizures or a child with abnormal developmental history, or a child with a family history of epilepsy, have a risk of epilepsy development of about 5 to 10 percent after a febrile seizure. Some other factors related to the risk of epilepsy, if that first seizure is complex, the risk of epilepsy is about 18 times that of simple febrile seizures.

If the child has focal seizures, prolonged seizures, and repeated episodes within 24 hours during the same illness, the risk of epilepsy is 2%, 7%, 20 percent and 50 percent was 3, or all of those risk factors. One cohort study of almost 200 children with febrile seizures found the risk of epilepsy was highest in the first five years and appeared to decrease over time.

And other risk factors for epilepsy that have been identified in independent studies include Todd’s paralysis, short fever duration before the seizure, late onset of febrile seizures over three years of age, and multiple febrile seizure recurrences. There’s a tenfold increase. So what’s the evaluation and management for simple febrile seizures?

I joke that it’s discharge home, but really, Choosing Wisely recommends that we do not order laboratory studies or CT scans for a patient with a simple febrile seizure who has returned to baseline mental status. Labs just aren’t necessary. This postictal period is usually brief, so 20 to 30 minutes or up to 2 hours.

I think 2 hours is too long. And if the kid returns to a neurologic baseline, they’re unlikely to have a metabolic or structural abnormality that you’re going to need to identify. So in that child who has recovered with a normal neurological examination, You don’t need labs. They’re painful, they can give you erroneous or unexpected or irrelevant results, and they’re expensive.

So really focus on targeted testing. If you think they’re at risk for a UTI, well, yeah, get a urine. Go after COVID and flu if you think the swabs are beneficial or strep, but otherwise, you can avoid labs. In general, I think parents are most worried that their child’s gonna have a brain tumor or something wrong inside their head, and naturally, their minds will gravitate towards getting a CT scan.

These are expensive studies with a large amount of radiation, and in the absence of concerning signs on history in the exam, the rate of scarring abnormalities, mass, stroke, or other problems is really, really low, so like less than 1%. So in general, you don’t need a CT scan to show that the brain looks normal.

Children with recurrent febrile seizures or epilepsy following febrile seizures benefit more from MRI and EEG. What about complex febrile seizures? How do we evaluate and manage them? So again, focal onset greater than 15 minutes and or recurrent within 24 hours. The majority of children who develop complex febrile seizures will do so with their first seizure.

Todd’s paresis, so transient hemiparesis following a febrile seizure, usually of a complex or focal type, is rare and happens in about 1. 5 to 2 percent of cases. Prolonged or focal febrile seizures have a higher likelihood of meningitis or structural abnormalities, but still that risk is low. And so even in complex febrile seizures, if the child recovers, EEG or MRI may be the only test, if any, that they need.

You should develop a specific plan for each patient with each patient. A pediatric neurologist. So in terms of lumbar puncture, yes, you can get an LP and CSF studies to exclude meningitis or encephalitis in a child with a complex febrile seizure. The literature has long told us that in children older than 6 months of age who are completely vaccinated, you do not need to get an LP and CSF.

to rule out meningitis in a child with a simple febrile seizure. Admittedly, 25 percent of children with meningitis will have seizures at or before the initial presentation, but almost all of these kids will also have other signs and symptoms of meningitis, like altered consciousness, nuclear rigidity, a petechial rash.

So if you have a child with a complex febrile seizure, but they don’t have any other signs of meningitis, you don’t necessarily need to tap them. If febrile seizures occur after the second day of illness, if you have febrile status epilepticus, these should be other considerations as to whether or not an LP is needed.

But overall, the yield of LP is very low. Now note that pleocytosis can be seen in epileptic seizures, but it’s actually rare in febrile seizures without meningitis. So what does the AAP say? Well, they say that LP should be performed when there are meningeal signs or symptoms or other clinical features that suggest possible meningitis or intracranial infection.

Yeah, you should consider it in infants between 6 and 12 months of age if the immunization status for Hib or strep pneumonia is deficient or undetermined. So, 3, Strep pneumos, and two or three HIBs. And you should also consider an LP when the patient is on antibiotics, because antibiotic treatment could mask the signs and symptoms of meningitis.

This is perhaps the most nuanced scenario. If a child has a complex febrile seizure, and fortunately it’s a rare one that you’ll see, but you should consider, maybe they’re on otitis media, and then they have a febrile seizure, and it’s complex, probably tap that kid. In a study from Kim published in Pediatrics in 2010, they looked at 526 children with complex febrile seizures.

Almost two thirds of this population got lumbar punctures and only three had meningitis, all with a reason to suspect it. So one was clinically non responsive, one had a bulging fontanel and apnea, that’s a bad combo, and one was well appearing but had a positive blood culture for strep pneumonia and they didn’t do an LP, so they just presumed that they have meningitis?

I’m squinting. You can’t see that on the podcast, but that kid probably didn’t have meningitis. Another relatively large cohort of children with complex febrile seizures, published in 2017, showed that the incidence of bacterial meningitis in 839 patients with complex febrile seizures was 0. 7%, and none of them had HSV.

All five of those patients with meningitis had a concerning exam, and four out of five were less than 12 months of age. So, if somebody with a complex febrile seizure is going to have meningitis, there’s going to be other stuff going on. Simple febrile seizures do not require neurology consults or admissions.

They can be discharged home. Previously healthy and developmentally and neurologically normal children with two brief self resolved seizures within a 24 hour period, so technically a complex febrile seizure, can be discharged home with as needed neurology referral if the family and you are comfortable with that plan.

For Febrile status epilepticus, you should stop the seizure with medicines and admit to neurology in the PICU. And complex febrile seizures with focal features, strongly consider admission and always discuss with child neurology. And so briefly, let’s talk about that neurology referral and follow up.

Neurology will often see children in the near term with complex febrile seizures who you felt are safe for discharge but need evaluation. EEG itself is not useful in determining the risk of recurrent febrile seizures. If you’re looking for epilepsy, abnormalities are more likely to be seen on EEG when it’s performed shortly after the seizure, so less than 10 days, and when convulsions are of a longer duration and have focal features.

In children with focal complex febrile seizures, neurology is almost always going to get an MRI as well, and in children under 6, they’re probably going to need general anesthesia to do that. Alright, so what about the management of febrile status epilepticus? So originally this was defined as greater than 30 minutes.

It’s back down to 15 minutes, but there’s a current movement to define status epilepticus as greater than 5 minutes. And 5 minutes is a really long time to watch a kid seize, so I get it. In 1 third of febrile status epilepticus, the actual seizure duration is underestimated in the emergency department.

And the clinical clues that a seizure has ended are often subtle. So a child that is no longer seizing will have closed eyes and deep breathing. If the eyes are persistently open and deviated, even if there’s no limb convulsions or stiffening, they may have ongoing focal seizures. It’s really hard to figure this out.

I’ve also seen kids that are febrile having rigers, or just from a sympathetic surge after a seizure. So, response to painful stimuli, closed eyes, regular breathing. These are all subtle findings along with your vitals like capnography that can help you figure out if that kid is still seizing. In a wonderfully named study called Febstat, which was initially published back in 2008 as a multi center perspective cohort of 119 children one month of five years with febrile status, they noted that the median duration of seizures was 68 minutes.

They were convulsive in all but one child. They were continuous in half and intermittent in the other half. Two thirds of these status. Patients were partial. It was the first febrile seizure for 3 out of 4 children in the study. And HHV 6 was the most common identified infectious etiology. There was also a higher than expected family history of epilepsy in this population.

So if the seizure is going on longer than 5 minutes, start with an IV benzodiazepine if you can. So diazepam or lorazepam. Buckle midazolam or rectal formulations like diastat are alternatives if you don’t have an IV. If that first benzodiazepine doesn’t work, give it again at five minutes. If that doesn’t work five minutes later, give a second line drug.

Generally, levotiracetam or Keppra is the first choice for second line, but you could use fosfenitoin or valproate if you’ve got them. So are febrile seizures associated with an increased risk of mortality? This is very pertinent to familial concerns. Early reports actually suggest that febrile seizures were associated with an increased risk of sudden death later on.

We found that that’s probably not true and that small excess in mortality is really restricted to complex febrile seizures. These patients have pre existing neurologic abnormalities. Those are the ones that are really most at risk. Alright, well what about prescribing preventative medicines or rescue drugs?

I don’t think that children that have a single simple febrile seizure need to be prescribed rectal diastats. But if they’ve had a prolonged febrile seizure, including febrile status epilepticus, or have had multiple febrile seizures, prescribing diazepam rectal gel or midazolam nasal spray in an older child who is an appropriate size could be a good idea.

One dose administered rectally or nasally will not lead to respiratory depression. And so in general, if you’ve got a child that’s at risk for a prolonged future febrile seizure, are good candidates for rescue meds. through a process of shared decision making. Now, you could prevent the risk of subsequent febrile seizures by putting a kid on prophylactic anti epileptic medications.

Most febrile seizures are benign and the side effects of the AEDs generally outweigh the benefits. So you don’t need to put somebody on phenobarbital to stop them from having another febrile seizure. So the use of antipyretics, so acetaminophen, ibuprofen, at the first sign of fever does not prevent a recurrence of febrile seizures in a child that’s had one before.

Morata and colleagues did a single center perspective randomized control trial back in 2018 that noted that regular antipyretics may reduce the recurrence of febrile seizures during the same fever episode, so during that illness. But other studies, including one from Rosenblum back in 2013, which was a meta analysis of three RCTs of acetaminophen, ibuprofen, and diclofenac, starting antipyretics, at the onset of illness could not reduce the rate of recurrent febrile seizures compared with placebo.

Why don’t antipyretics work? Well, they facilitate heat loss, but they don’t inhibit heat production, or lower the threshold convulsive temperature during the initial stage of fever that triggers a seizure. Now, interestingly, phenobarbital can actually treat fever and seizures, but, you know, there’s side effects, so it’s not recommended.

Okay. So is there anything else on the differential? If you’re sure it’s a febrile seizure and you know what you’re doing, generally, you’re right. But, kids can have shaking chills, which are involuntary movements in febrile children that are fine, rhythmic, and oscillatory movements about a joint. They rarely involve the facial or respiratory muscles.

They usually involve both sides of the body simultaneously, and they are not associated with the loss of consciousness, and they are suppressible by touch. So shaking chills can easily be differentiated from fevers. Children with breath holding spells, the cyanotic or pallid types, will sometimes have stiffening or convulsing when they lose consciousness.

And yes, I’ve seen kids with colds and fever who have breath holding spells. And then there’s genetic epilepsy plus febrile seizures, which you are not going to diagnose in the ED. These are autosomal dominant seizures. mutations of sodium and calcium channels that lead to seizures with fever in early childhood that continue beyond six years of age.

And then there’s Dravet syndrome. So, Dravet syndrome is also known as severe myoclonic epilepsy of infancy. It will often resemble complex febrile seizures under one year of age. It’s a de novo mutation, so not inherited, of a voltage gated sodium channel in more than 80 percent of the patients with it.

So that’s why phosphenytoin doesn’t work in Dravet syndrome. And if you’ve heard of Dravet, probably the only thing you remember is that phosphenytoin doesn’t work. And I’ve been told by a wise pediatric neurologist where I work that Any female patient under 12 months of age with complex febrile seizures has Dravet syndrome until proven otherwise.

So that’s one special population with a complex febrile seizure, even two in a 24 hour period that deserves special workup. So let’s end with the last and perhaps most important question. How do we talk to families about febrile seizures? Well remember, they are scared, right? I would acknowledge that this was perhaps the most frightening thing they’ve ever seen their child do.

They are worried that it’s not going to stop and that their child would die. They may have felt helpless, but reinforce what they did right. Maybe they moved their child to the floor in the rescue position, or quickly called 911, or got help. All of these are active decisions that the family made to help their child, even if they could not have prevented the febrile seizure.

Define what a seizure is in ways they’ll understand, including how common febrile seizures are. Again, simple febrile seizures, 1 out of 50 children. Explain how the body protects itself during seizures. So there’s a sympathetic surge leading to increased heart rate. The skin will look pale due to peripheral vasoconstriction and shunting of blood to the core organs.

And when you’re seizing, you will close your glottis, your vocal cords, to prevent from aspiration, which leads to perioral cyanosis. So parents often will recall their child stiff, convulsing, and blue in the face. All of these are physiologic things that we expect during seizures, and I think it’s important to address how those were things that the body did You are certainly going to want to say whether or not the child had a simple or complex febrile seizure, because that will dictate what you do next.

Discuss the recurrence risk and what to do if it happens again, i. e., one third after you’ve had one, 50 50 chance after two or more, and you should always be evaluated by default. Talk about the use of antipyretics and their limited impact on recurrence. So they could reduce the risk during this illness, but they don’t necessarily reduce the risk in a future febrile illness.

And use lab tests, and especially CT scans of the brain judiciously. In a child who has recovered, is back to their baseline, has a normal neurologic exam, and no underlying neurologic problems, they’re unlikely to recover. to have any central nervous system abnormalities seen on imaging, nor any significant metabolic or infectious abnormalities seen on targeted lab testing.

So it’s A OK to not get any studies, but remember, you’re not doing nothing. You’re providing education and reassurance to a worried family. You can do this. If the child has recovered and you think it’s a simple febrile seizure, even if you’ve never seen one before, you’re gonna be right. You They’re that common.

I definitely recommend that you practice your speech or approach to febrile seizures before you go into the room if you haven’t done this before. So find an experienced senior resident, fellow, or attending and review it with them. You want to make sure that you can both give the family useful information.

but also not overwhelm them and anticipate what questions they might have. Well that’s it for this episode focused on febrile cedars. I hope you found it useful and will take the information back with you to your next shift in the emergency department. If you have suggestions for other topics that you’d like me to address in the future, send them my way.

I’ll take an email, a comment on the blog, a message on a social media platform of your choosing. My 12 year old told me that I should remind you to subscribe, and and review. Bottom line is I’m just happy if more people listen because that means more people learn and any feedback you can send in my direction, even if it’s in the form of a review, is very welcome.

For PEM Currents, the Pediatric Emergency Medicine Podcast, this has been Brad Sobolewski. See you next time.

This episode will help you better prepare for and manage children with inborn errors of metabolism in the Emergency Department. Consider it a supplement to what you remember from Biochemistry and the instructions on the family’s laminated care plan sheet. My special guest podcaster, Emily Groopman, is an actual Pediatric Geneticist in training and we hope that you will find this episode useful.

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Emily Groopman, MD, PhD

Emily Groopman, MD, PhD is a first-year resident in the Combined Pediatrics-Medical Genetics Residency Program at Children’s National Hospital/NIH. She did her MD/PhD at Columbia University, where she investigated the diagnostic utility of exome sequencing for kidney disease. She is a member of the Clinical Genome Resource Inborn Errors of Metabolism (IEM) Clinical Domain Working Group, where as a biocurator she assesses the pathogenicity of variants in IEM-associated genes to facilitate expedited genetic diagnosis for IEMs. She aims to become a physician-scientist in pediatrics and medical genetics, engaging in bench-to-bedside research that utilizes multi-omics-based approaches to provide a molecular diagnosis and support personalized care for individuals with suspected rare genetic diseases and their families. You can contact her via email at [email protected].

References

Jeanmonod R, Asuka E, Jeanmonod D. Inborn Errors of Metabolism. [Updated 2023 Jul 17]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK459183/#

Rice GM, Steiner RD. Inborn Errors of Metabolism (Metabolic Disorders). Pediatr Rev. 2016 Jan;37(1):3-15; quiz 16-7, 47. doi: 10.1542/pir.2014-0122. PMID: 26729777.

Burton BK. Inborn errors of metabolism in infancy: a guide to diagnosis. Pediatrics. 1998 Dec;102(6):E69. doi: 10.1542/peds.102.6.e69. PMID: 9832597.

Transcript

Note: This transcript was partially completed with the use of the Descript AI

Welcome to PEM Currents, the Pediatric Emergency Medicine Podcast. As always, I’m your host, Brad Sobolewski, and this episode focuses on the management of children with metabolic disorders who present to the emergency department. I know that this is a subject that makes us all a little bit nervous, and you’re just hoping that the parents have a good laminated sheet to tell you everything that you need to do.

Unfortunately, that’s not always the case. And, let’s face it, there are some great principles that you can apply across metabolic diseases to make sure that you are safely taking care of these children. And you’re thinking, hey! Brad’s not a pediatric medical geneticist. No, I’m not. So I called in a ringer.

Or, well, the ringer called me. So my special guest host on this episode is a trainee in pediatrics and medical genetics. Her name is Emily Groopman, and she’s a current resident at Children’s National Hospital. After doing her MD PhD at Columbia University, where she investigated the genetic diagnosis of kidney disease, she started her residency training with the long term goal of being a physician scientist caring for patients with rare genetic disorders.

She came to me with the idea for this episode based on a recognized need to reinforce key principles in the management of children with inborn errors of metabolism who present to the emergency department. I put a lot more information about Dr. Groopman and how to contact her in the show notes. But now I’m going to pass the mic.

Take it away, Emily.

Inborn errors of metabolism, or IEMs, refer to a diverse group of disorders that result from mutations in genes that are involved in pathways responsible for breaking down nutrients and generating energy. In other words, metabolism. While each of these conditions is individually rare, when considered as a group, they are IEMs occur in approximately 1 in 2, 500 births and can have severe health consequences, including ketoacidosis, cardiac arrhythmias, and encephalopathy.

Therefore understanding these diseases, their presentations and their evaluation is critical for emergency medicine providers. So first, a little bit about their etiology and epidemiology. IEMs are primarily caused, as I mentioned, by mutations in genes involved in metabolism. In other words, genes that include enzymes and other proteins that are involved in breaking down nutrients like carbs, proteins, and fats, and generating energy.

IEMs vary in their inheritance. Most IEMs are inherited in an autosomal recessive manner. Meaning that an individual must inherit two copies of the mutation, so one from each of his or her parents, to be affected. Since an individual must have two copies of the mutation to be affected, the parents and other family members who have one copy, which are known as carriers, will be unaffected.

So, importantly, you may not have a positive family history. Other factors, such as environmental influences, epigenetic changes, the microbiome, and additional genes, may also impact the penetrance of IEMs. In other words, whether or not individuals with a disease causing mutation manifest the associated genetic disease, and also the expressivity.

In other words, which features of the disease individuals with the mutation show for these conditions. Now, newborn screening, or NBS, includes testing newly born infants for certain IEMs.

Since which IEMs are tested for vary state by state, the tests used do not, and the tests do not have perfect sensitivity. And not all IEMs are included on NBS, NBS can miss individuals with IEMs. Therefore, and I want to stress this again, negative results on NBS do not rule out the possibility of IEM.

And it’s always worth considering IEM among children, including among older children and teens, who present with suggestive symptoms. So what’s the pathophysiology of IEMs? Now, IEMs result from disruption of major metabolic processes in our body. And these major metabolic processes include carbohydrate metabolism, protein metabolism, fatty acid oxidation, and glycogen storage.

And together, these processes help store us store nutrients from the food we eat and use it to generate energy. Now, carbs are our body’s preferred source of energy. When we eat, our bodies break down carbohydrates into glucose, which can be used by our cells to generate energy, aka ATP, via cellular respiration.

The glucose that is not immediately used to generate energy is stored in the liver and muscle cells as glycogen. When we’re between meals, in other words, we’re not eating, we’re not fasting, our bodies break down glycogen into glucose so that we can continue to generate the energy our cells need to function.

And altogether, we have enough stored glycogen to last for approximately 24 hours without food. Now, let’s say you fasted for that 24 hours, and at this point your glycogen stores will be depleted. At this point, our bodies have to shift to alternate pathways, first going down the hierarchy of gluconeogenesis, where you can make glucose from amino acids and other non sugar compounds, and then fatty oxidation.

In other words, breaking down fatty acids into the compound acetyl CoA, which can be used to generate energy. Now importantly, fatty oxidation yields ketone bodies. And when the body is in a state that it’s relying primarily on fatty oxidation to generate glucose. You’ll need to get, you’ll accumulate high amounts of ketones leading to ketoacidosis, which is a metabolic emergency.

Now IEMs can disrupt any of these pathways and importantly can have severe health consequences. So what are you going to see? On clinical presentation. Now, first off, realize that most IEMs present with very nonspecific clinical features. You won’t be able to diagnose them on history and physical alone.

And biochemical testing is really needed in most cases to independently diagnose a specific IEM. Therefore, in the ED, the goal is really to recognize the science and symptoms on history and physical exam that are suggestive of metabolic disease. identifying which specific IEM the patient has is part of the later long term evaluation, typically with the help of your friendly geneticist.

It is not the job or the expectation of the EM provider. So what are some of these suggestive clinical features? They include neurologic dysfunction, which is one of the most common that includes things like developmental delay, regression, AKA loss of developmental milestones, hypotonia, encephalopathy, or seizures.

GI symptoms are the second most common, and they include vomiting, food intolerance, food aversion, GERD, refractory to normal antireflux measures, diarrhea, and dehydration. You should also think about IEM in cases where you have failure to thrive, exercise intolerance, or autonomic instability. Now, as I mentioned earlier, since these are autosomal recessive disorders, where you need to have two copies of the mutate, of a mutation to manifest disease, oftentimes family history is negative.

However, sometimes you might hear of siblings or other relatives who had early onset neurologic or GI dysfunction or died early in life, and this can often be attributed to sepsis or sudden infant death syndrome because the symptoms of these overlap with IEMs. You also might see a family history of multiple miscarriages and or constant infinity.

Now typically, IEMs involved in glucose, protein, or fat breakdown, which are, the formal term for them is called intermediary metabolism, will have a short asymptomatic interval. They would kind of like there’s a honeymoon period of days to weeks depending on the IEM after birth. And then they’ll present with acute metabolic decompensation in the neonatal period.

And these neonates typically present looking really, really unwell. So they’re lethargic, they might vomit, they’re hypotonic, hypothermic, they might have fever or seizures. And this is due to buildup of the toxic intermediates of the stalled metabolic pathway. Now the important thing for EM providers to know is that this can mimic the presentation of sepsis.

So you should consider IEM on your differential, especially when the ID workup is negative. And the neonate’s symptoms are refractory to standard measures. In these children, in children, IEMs can present with acute metabolic or neurologic decompensation, like vomiting, coma, or seizures, oftentimes precipitated by episode, things that are metabolically stressful.

So think infection, exercise, or change in diet. Now IEMs involving excretion pathways will generally present with symptoms related to the buildup of the toxic metabolites that cannot be excreted. Now, this, because this gets a lot of buzz, hyperammonemia is a very common feature of a number of different IEMs.

And so it’s important to know its presentation. Hyperammonemia presents with difficulty feeding, lethargy, altered mental status, seizures, vomiting, and vital symptoms of anomalies, most commonly loss of regulation or low core temperature. Now, in contrast, individuals with IEMs that involve pathways for accessing stored energy Can be asymptomatic or well appeared for long periods of time as long as they have a steady supply of energy.

So for instance, in infants who often follow a regular feeding schedule, they can slip under their radar as they’re getting enough energy and in a period in routine forms, and they don’t need to then have any kind of tapping into their stored energy. But again, metabolic GI illness, interrupt other interruptions in feeding schedule, intense exercise.

will result in symptoms. And depending on the specific IEM, these can range from severe metabolic decompensation like hypoglycemia or ketoacidosis, to more subtle features like muscle cramps. So to summarize, consider IEM for neonates with severe unexplained progressive or refractory illness shortly after birth, children who have severe neurologic or GI dysfunction, neglects associated with vomiting.

For metabolic stressors like fever or fasting, and children who are presenting with acidosis or hypoglycemia. Now, what should we do for evaluation and next steps in management? So, again, to reinforce, since IEMs have very specific non specific presentations, the goal in the ER is not to specifically diagnose the IEM.

Rather, it’s recognizing the child in front of you may have an IEM and do what you need to do to acute, for acute stabilization for their associated symptoms. So first, like pretty much many presentations, do ABC, get your PALS as indicated, and get IV access. Next, you want to stop the intake of potentially toxic compounds like protein, fat, glucose, and fructose, and this includes NG or G tube feeds if the child does have them.

Make them NPO and give IV, and give IV fluids with 10 percent dextrose, normal saline, or half normal saline. So D10NS or D10 half NS at one and a half times their maintenance rate. And the goal here is to give glucose, which is that, you know, number one, pure substrate for energy iteration metabolic pathways at a sufficient volume or rate so that this patient does not need to use the other metabolic pathways that might be causing their presentation.

Next, get stat labs, look at metabolic anomalies for blood labs. You want to get some lights, you can get a BMP or CMP glucose, LFT, CRP. CK, urea, and also assess their acid base status, so venous, capillary, or arterial blood gas, and also get COAGs. you want to look at their ammonia and lactate and importantly, if you can, , you want to get a plasma sample for some more sophisticated metabolic tests that can be done later.

So those would be a plasma sample for plasma amino acids, organic acids, acyl carnitine, other compounds, which your friendly geneticist, when you consult them will be incredibly happy you got. Now you also want to get some urine samples. You want to check the color and odor with a urine analysis. Look at the pH, whether there’s glucose, protein, ketones in there.

And you can also store some urine sample for downstream testing. As certain metabolic disorders, you want to look at organic acids in the urine. Now, if you end up needing to get an LP, you can also freeze some extra CSF for downstream testing. Aim for around two to five mils. And then, aside from these tests, There are some additional studies that might be indicated by clinical symptomatology.

So for instance, if they’re having cardiac issues, think about getting an EKG or an echocardiogram. If they’re encephalopathic, you want to consider neural imaging, CT or MRI. And importantly, call a stat genetics consult for further guidance or management. These patients typically do need to be admitted, even if it’s just for monitoring, and if they’re very much deranged in their ABCs, their mental status, they may need to be admitted to the ICU.

So you’ve done your initial workup, nothing’s really conclusive yet, and this patient’s still in the ED. your genetics consult hasn’t responded yet. What should you do? First, continue the glucose infusion. Then, once you get the go ahead from your genetics consult, send samples for specialized metabolic evaluation, including plasma amino acids and acyl carnitines, urine amino acids and organic acids, and whatever else your consult recommends.

Keep an eye on their labs. Again, your consult can give you some helpful tips on the frequency of monitoring, including their lights, glucose, lactate, acid base status, and ammonia. And importantly, if you’re at a referring facility, the most important things are really the basics. So get the ABCs, start D10.

If the patient has a metabolic plan, whether it’s in their electronic medical record and or in their carrier gibbous fans. Follow it. This was made by people who know them very well. If labs are very difficult to get, let’s say the kid’s a difficult stick, at least get a finger stick glucose, get IV access, and start those fluids, D10, either NS or half NS, at one and a half times maintenance.

An obtundate or somnolent child can still tolerate intraosseous access, especially if you put the lidocaine in, 0. 5 mg per kg, max 20 mg. You can use 1 or 2 percent Lido. this video. And really get that access in so you can start those fluids and stabilize the child. Make plans to safely transport the child to a tertiary care after stabilization.

and connect and contact genetics and the accepting ED as soon as you can. Emily, thank you so much. I really appreciate you sharing your knowledge and information and hopefully this was a helpful refresher and primer for the next time that you see a child with a metabolic disorder in the emergency department.

If there’s other topics that you want to hear on the podcast, reach out and let me know. I will take an email. I will take a direct message on X. I will take a comment on Facebook or the blog. If you have the time, leave a review. It helps more people find the show, and therefore more people learn about the care of ill and injured children in the emergency department.

And if you’re like Dr. Groopman, And you’re wondering, Hey, can I record a podcast? The answer is yes, you can. If there’s a topic that you’re interested in learning and teaching about, and it relates to the care of children in the emergency department, send it my way. For PEM Currents, the pediatric emergency medicine podcast.

This has been Brad Sobolewski. See you next time.

Newborn infants need intramuscular injections of Vitamin K in order to produce critical clotting factors. If they don’t get it they can have potentially life threatening bleeding.

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References

• American Academy of Pediatrics, Committee on Fetus and Newborn.  Controversies Concerning Vitamin K and the Newborn.  Pediatrics 2003 July; 112(1):191-2.
• Ross, JA, Davies SM. Vitamin K prophylaxis and childhood cancer. Med Pediatr Oncol. 2000 Jun;34(6):434-7.
• Cornelissen, M., et al.  Prevention of vitamin K deficiency bleeding: efficacy of different multiple oral dose schedules of vitamin K.  Eur J Pediatr.  1997 Feb; 156(2):126-30.
• Greer, FR, et al. Improving the vitamin K status of breastfeeding infants with maternal vitamin K supplements. Pediatr. 1997 Jan;99(1).
• Kher P, Verma RP. Hemorrhagic Disease of Newborn. [Updated 2023 Jun 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK558994/#

Transcript

Note: This transcript was partially completed with the use of the Descript AI

Welcome to PEM Currents, the pediatric emergency medicine podcast. As always, I’m your host, Brad Sobolewski. Today, we’re gonna talk about vitamin k deficient bleeding, also known as hemorrhagic disease of the newborn. This is a bleeding disorder that manifests in the first few days to weeks of life after delivery. Under the umbrella are a whole range of hemorrhagic diseases, but the most important is vitamin k deficient bleeding.

I’ll get into why in a moment. Vitamin k itself is a fat soluble vitamin mainly synthesized by gut bacteria. Newborns have minimal vitamin k reserves in a sterile gut. And there’s insufficient placental transfer and breast milk is deficient in vitamin K, so that’s why infants need vitamin K at birth. Without it, they can’t produce clotting factors 2, 7, 9, and 10.

You need all those. In brand newborns, the levels are about 20 percent or less of adult values, but within a month after birth, they arise to within normal limits. Other causes of hemorrhagic disease of the newborn include hereditary clotting factor deficiencies such as hemophilia A or B. And the most common item on the differential, especially for late onset, which we’ll talk about in a moment, is trauma, non accidental or accidental trauma. So why am I covering this topic?

Well, a lot of people out there are actually refusing vitamin k for their newborns. Why? Well, families state that they have concerns about the preservative in the injection, maybe that it could cause autism. It doesn’t. The pain from the injection could be harmful to the infant.

They perceive that the intramuscular vitamin k is a vaccine. It’s not. The dose of intramuscular vitamin K is too high. It isn’t. A potential for adverse reactions to an injection like anaphylaxis.

Anaphylaxis can happen after IV infusion and it’s been rarely reported after I’m injection, like winning the Powerball odds. The injection is perhaps a potential entry for germs, that the intramuscular vitamin K causes cancer. So there was 1 study published in the British Medical Journal in 1990. It raised that concern, suggesting that the risk of cancer was doubled in babies that receive vitamin K after birth. Many studies since then in Europe and the United States have refuted this claim and there is absolutely no association between vitamin k and cancer.

Other concerns about vitamin K include that vitamin K may overwhelm the newborn’s immune system. There’s just a general desire to be natural and perhaps a belief that oral vitamin k prenatally to the mother is more effective, but it isn’t. Furthermore, parents who refuse IM vitamin k tend to refuse other preventative measures, including the Hep B vaccine at birth, prophylaxis against gonococcal ophthalmia, which is really bad, and subsequent routine vaccination. Approximately 1 half of the severe cases of vitamin k deficient bleeding are associated with parental refusal vitamin k during the birth and hospitalization. So hemorrhagic disease of the newborn vitamin k deficient bleeding can be categorized into 3 groups based on the age of onset.

Early occurs within the first 24 hours after birth and it’s generally due to maternal medicines that block vitamin k action. Uh, most commonly, these are anti epileptics like phenytoin, phenobarbital, carbamazepine or primidone. They could also be anticoagulants, coumadin, aspirin or even some antibiotics like cephalosporins. The incidence in infants who have not received vitamin k prophylaxis in parents that are on these medicines could be 6 to 12 percent. Classical vitamin k deficient bleeding happens within 1 week of neonatal life, the second through the seventh day.

With vitamin k, the risk is 0.01 percent. If babies are exclusively breastfed and they don’t get vitamin k at birth, that increases the risks. Late onset is from 8 days up to 6 to 12 months. And this is generally exclusively breastfed babies and babies with diarrhea, cholestasis or malabsorption because vitamin k absorption is dependent on bile. The risk is about 1 in 15000 to 1 in 20000 births.

Most common symptom of late onset is intracranial bleeding with a mortality of 20 to 50 percent and all the associated morbidity of an intracranial hemorrhage. The reason for the increased risk in exclusively breastfed infants, I. E. Even those who don’t get any solids or anything else, is because there’s only marginal levels of vitamin K in breast milk. Other causes of late onset, cystic fibrosis, celiac, chronic diarrhea, alpha 1 antitrypsin deficiency, and forms of hepatitis.

So if you suspect vitamin k deficient bleeding, take a good history. These are some of the points in the history that could lead to you making the diagnosis. So take a history of the drugs that mom was on during pregnancy, especially anticonvulsants. Preterm babies are at a higher risk. Breastfed or bottle fed?

Again, bottle- or formula fed infants are at a lower risk because fortified feedings have higher levels of vitamin K. Where was the delivery? Home delivered infants don’t have access to immediate vitamin k prophylaxis at the same rates that hospitalized infants do. So physical findings that you might see in a patient with vitamin K deficient bleeding include cephalohematoma, intracranial bleeding, intrathoracic bleeding, like hemoptysis or associated respiratory distress, intra abdominal bleeding, so you can see melena, hematemesis, you know, isolated GI bleed. You know, you could also think intussusception and mccals.

Skin, you’ll see petechiae on the mucous membranes. You’ll see hemorrhage or petechiae inside the mouth, on the gums, in the nose, excessive bleeding after circumcision, bleeding from the umbilical cord stump after it’s cut and if the umbilical cord falls off, bleeding from vaccine sites. And I mentioned it before and I’ll say it again, but intracranial bleeding is the worst possible outcome. It’s associated with late onset vitamin k deficient bleeding, and it presents with a floppy baby, lethargy, feeding difficulties, bulging fontanels, poor respiratory effort, altered consciousness, convulsions or pallor. These are sick looking babies.

So in evaluation, you wanna get a CBC. Uh, vitamin k deficient bleeding will have normal platelet levels. Thrombocytopenia actually suggests a maternal immune thrombocytopenia in a newborn. They can make antibodies to platelets which can cross the placenta. Clotting profile, the INR will be greater than 4, because again those factors are needed for proper blood clotting.

The PT will be more than 4 times normal. That’s increased due to decreased activity of factor 7. The PTT will also be increased due to decreased activity of factors 2, 9, and 10. The clotting time will be increased due to clotting factor deficiencies, but fibrinogen levels will remain normal. Protein induced by vitamin k antagonists, PIVCA, I guess.

There’s an estimation you can get a lab on that. Any amount of PIVCA is abnormal and indicates vitamin k deficiency. This disappears around day 5 after the administration of vitamin k, but this lab is not part of the routine ED evaluation. Imaging is targeted at the differential diagnosis in the site of bleeding. So get a chest x-ray or an ultrasound, determine if there’s bleeding in the body cavities, you know, the chest or the abdomen.

Um, CT and MRI are most useful to evaluate for intracranial hemorrhage. So treatment. Uh, vitamin k at birth. I think I mentioned that before. So for an infant that’s greater than 1500 grams, so most of the babies that you’ll be taking care of, 1 milligram I’m Less than 1500 grams, 0.3 mgs per kg up to 0.5 mg per kilogram I’m Intravenous vitamin K is not recommended for prophylaxis in preterm infants.

The form that we now give is vitamin K1, It’s a naturally occurring fat soluble form of vitamin k. So before the introduction of vitamin k 1, long before any of us trained, they used vitamin k 3. K3 was a synthetic water soluble derivative. And in higher doses, it was associated with kernicterus hemolytic anemia and hyperbiliruminemia. So vitamin K1, current version, very safe.

Again, in the US, intramuscular vitamin K at birth is recommended. There are no known toxicity or side effects associated with vitamin K1. Now in some parts of Europe, they’ll do oral regimens at birth, at 2 to 4 weeks, and at 6 to 8 weeks. Uh, they can be weekly or even daily. There’s no licensed oral form for newborns in the US.

Some have given infants the injectable liquid by mouth, but it’s not observed and that’s an unstudied intervention. There’s no safety or efficacy data available on that route of administration. In countries that have gone to oral prophylaxis, failures, even with good compliance, have been reported. Failures have not been reported with routine I’m prophylaxis. So based on the available observational evidence, a single I’m dose of vitamin k appears to be more effective in preventing late onset vitamin k deficient bleeding versus oral regimens.

So maternal dietary changes have little effect overall on vitamin K status of the newborn. There was 1 smaller study that showed that 5 milligrams a day or 800 percent of the recommended daily allowance may raise infant serum levels to near formula fed infants in moms that are breastfeeding. But there’s no FDA approved multivitamin that contains that amount of vitamin K. So if you have a baby with hemorrhagic disease of the newborn, in early and classic forms, the treatment is oral vitamin K, 2 milligrams dose, repeated at 2 to 4 weeks and 6 to 8 weeks. And so again, these are milder forms of bleeding.

All breastfed babies with diarrhea and malabsorption situations require an additional postnatal dose of vitamin K to prevent late onset vitamin K deficient bleeding. For the late form of the disease, oral vitamin K is not as efficacious as parenteral. Hence, the 0.5 to 1 milligram single I’m dose should be administered. A presumptive diagnosis of vitamin k deficient bleeding should be made in an infant presenting with bleeding or neurologic symptoms, and either a prolonged PT and or INR, a history of not receiving vitamin k prophylaxis at birth. You should immediately give them 1 to 2 milligrams IV or sub q.

The vitamin k dose should normalize the coagulation profile within 2 to 3 hours. Infants may need resuscitation with blood products if they’ve lost more than 20 percent of their blood volume. And remember, a newborn can become hypotensive by bleeding enough inside their brain. And also, babies may need 10 to 20 ml per kilo of fresh frozen plasma. I’m going to leave you with a quote from Stanford University and Lucille Packard Children’s Hospital.

So the success of vitamin K prophylaxis has been so dramatic that many practitioners have actually never seen an infant afflicted with hemorrhagic disease of the newborn or vitamin k deficient bleeding. Now, it’s a popular trend in some areas to refuse prophylaxis in an effort to keep things natural for the infant. However, it’s important to keep in mind that the infants most at risk for the classic form of the disease are healthy babies who are exclusively breastfed. So we need to work closely with the parents who refuse vitamin k to help them understand the need for prophylaxis and the severity of the disease. The benefit of using I’m vitamin k injection should be explained to parents.

For those that refuse injection, counseling about the adverse effects of vitamin k deficient bleeding should be explained. The alternate oral dose of 2 milligrams should be recommended in the parents that strictly refuse I’m along with a repetition of that dose at 2 to 4 and then 6 to 8 weeks of age. Alright. So that’s all that I’ve got for this episode on vitamin k deficient bleeding AKA hemorrhagic disease of the newborn. Hopefully, you will feel armed to discuss vitamin k refusal with parents, as well as understand the different forms of the disease, including early, which is related to maternal medicines, classical, which is exclusively breastfed infants who don’t get vitamin k at birth, and the late form, which is the most dire and presents often with intracranial hemorrhage. If you have ideas for other episodes or topics you’d like to suggest, send them my way. I will take your feedback via email, a comment on PEMBLOG, a direct message on a social media platform, a snail mail.

However you wanna get feedback in my direction, let me know. Encourage your colleagues to listen to the podcast as well. More listeners means more learning. And, hey, I know that this can be a tough tough topic to discuss with some parents. I think we’re all better armed to have those conversations if we practice them beforehand.

So hopefully, this episode will prepare you for the next time you meet a newborn whose parents are using vitamin k. For PEM currents, the pediatric emergency medicine podcast, this has been Brad Sobolewski. See you next time.

This episode will help you recognize cellulitis and even differentiate it from erysipelas which is totally a different thing. You’ll also learn about treatment, whether or not a blood culture is necessary, and a whole lot more!

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References

Chen AE, Carroll KC, Diener-West M, Ross T, Ordun J, Goldstein MA, Kulkarni G, Cantey JB, Siberry GK. Randomized controlled trial of cephalexin versus clindamycin for uncomplicated pediatric skin infections. Pediatrics. 2011 Mar;127(3):e573-80. doi: 10.1542/peds.2010-2053. Epub 2011 Feb 21. PMID: 21339275; PMCID: PMC3387913.

Daniel J. Pallin, William D. Binder, Matthew B. Allen, Molly Lederman, Siddharth Parmar, Michael R. Filbin, David C. Hooper, Carlos A. Camargo, Clinical Trial: Comparative Effectiveness of Cephalexin Plus Trimethoprim-Sulfamethoxazole Versus Cephalexin Alone for Treatment of Uncomplicated Cellulitis: A Randomized Controlled Trial, Clinical Infectious Diseases, Volume 56, Issue 12, 15 June 2013, Pages 1754–1762, https://doi.org/10.1093/cid/cit122

Liu C, Bayer A, Cosgrove SE, et al. Clinical practice guidelines by the infectious diseases society of america for the treatment of methicillin-resistant Staphylococcus aureus infections in adults and children. Clin Infect Dis 2011; 52:e18.

Stevens DL, Bisno AL, Chambers HF, et al. Practice guidelines for the diagnosis and management of skin and soft tissue infections: 2014 update by the Infectious Diseases Society of America. Clin Infect Dis 2014; 59:e10.

Transcript

Note: This transcript was partially completed with the use of the Descript AI

 Welcome to another episode of PEM Currents, the Pediatric Emergency Medicine Podcast. As always, I’m your host, Brad Sobolewski, and today’s episode is all about cellulitis. What is it? Well when a break in the skin occurs, normal skin, flora, and bacteria can enter the subcutaneous tissue, where they do not belong, and they can also invade the lymphatic system.

And although this podcast episode is entitled cellulitis, I’m also going to talk about erysipelas. The two terms are not interchangeable. but both manifest as areas of skin, erythema, edema, and warmth. Cellulitis involves the deeper dermis and subcutaneous fat. Whereas erysipelas involves the upper dermis and there’s a more clear demarcation between the involved and uninvolved tissue.

There’s a fun fact, since the ear doesn’t have deep or dermal tissue, it’s always. ear-a-sipelas. I’ll pause for laughter. Anyway, a skin abscess, which is not the focus of this episode, is a collection of pus deep within the dermis or subcutaneous space. Impetigo, also not included in this episode, is a very superficial infection with that honey crusted drainage. There are also bullous versions. So cellulitis tends to develop in a bit more of an indolent fashion over a few to several days, whereas erys syphilis is more acute. You get systemic symptoms faster, such as fever. Chills, severe malaise, and headache. These can precede the onset of the local skin changes and start just in a matter of hours.

Clinically, for both, you’ll see areas of skin erythema. edema and warmth. You can also see petechiae and hemorrhage, as well as superficial bulla, vesicles, or even echemosis. Sometimes you also see regional lymphangitis or enlargement of the regional lymph nodes. If you’ve got a lot of edema surrounding the hair follicles, you can see some dimpling in the skin.

This creates an orange peel texture appearance, peau d’orange. I hope I pronounced that right. I took Spanish in high school. Anyway, the skin is warm to the touch, it’s uncomfortable, it hurts with movement, and some patients can describe an itchiness or a tight feeling in addition to the pain. You may see fever and other systemic symptoms, and cellulitis and erysipelas, especially in children, are nearly Always unilateral.

Bilateral red limbs? That’s probably something different. Complications that you should be aware of include bacteremia, endocarditis, septic arthritis, or osteomyelitis. Full blown sepsis and toxic shock syndrome. Fortunately, those are rare. So, in general, mild cellulitis has no systemic features in a patient with no significant comorbidities.

Moderate cellulitis has moderate swelling and tenderness with some systemic features like fever or tachycardia. Severe cellulitis has severe swelling and tenderness. really affecting function. It’s a larger body surface area, and you’ve got marked systemic features. So fever or hypothermia, extreme tachycardia, tachypnea, altered consciousness, a very unwell appearance, or even hypotension.

So what causes it? Well, bacteria, and the most common etiology. Staphylococcus aureus is actually an infrequent cause of cellulitis in children. But it can be seen more often in penetrating wounds. Methicillin resistant Staphylococcus aureus classically causes abscess formation. So you won’t really see that as the cause of isolated cellulitis or erysiplas in children.

So how do you make the diagnosis? Well, it’s clinical, right? Look for areas of skin that are erythematous, edematous, warm, and painful. Labs or imaging are not routinely necessary. If you think that there’s an abscess, you can diagnose it clinically by a localized area of induration or fluctuance or use an ultrasound.

Cellulitis can look like a cobblestone street on sonogram. And you should consider whether or not an abscess is present if you see significant induration, so thickening or hardening of the soft tissues, of greater than three centimeters or non uniform induration. The lesion’s been present greater than two to three days and changing or getting worse, and there’s a history of a previous incision and drainage in that patient.

And so do you need labs? Nah, not really. And the vast majority of patients of CBC or other labs will not aid in making the diagnosis of cellulitis. What about blood culture? Every febrile kid with cellulitis needs a blood culture, right? Not so fast, right? A blood culture can cost more than $200-300.

If you’re sending the kid home. Well, you definitely shouldn’t be sending a blood culture because if you’re worried about bacteremia and sepsis, that kid needs to stay in the hospital. And think about the risk of a false positive versus the risk of a true positive. So if the risk of a contaminant culture is greater than the risk of actually catching a bacteria, then don’t send it.

The cost of false positive cultures, repeat visits, length of stay, could be in the thousands of dollars. And so a lot of the previous studies on getting blood cultures were done in the immediate post Haemophilus influenzae B and Prevnar vaccine era. And we do now live in an era where these invasive organisms are fortunately not as big of a concern.

Vaccinate your children, people. But we do deal with MRSA. But still, for mild and moderate cellulitis, MRSA’s not really the etiology. And so even if a kid has a fever, But they look better after a dose of acetaminophen or ibuprofen. You don’t routinely need a blood culture. Now you could even admit a kid for IV antibiotics, maybe they’re dehydrated or they can’t take PO for some reason, without sending a blood culture.

So, admission does not mandate a blood culture. As always, you want to check with your local recommendations and follow algorithms present at your institution. So, what about disposition? So, with prompt identification and treatment with a correct antibiotic, which I’ll get to in a few minutes, patients can see an improvement in their signs and symptoms within about 48 hours.

The treatment failure rate is low, less than 1 out of 7, but probably a bit lower than that, with initial appropriate antibiotic treatment. Overall Cellulitis has a really good treatment prognosis. So when do you want to think about admission to the hospital or short stay unit versus discharge? Right, so you should probably admit a patient to the hospital if they have significant systemic symptoms.

You’re concerned about SIRS or sepsis. Again, fever alone does not necessitate admission. Especially if the kid looks better after antipyretics. If you are concerned that the child may need subsequent or future I& D, like they’re forming a phlegmon, they have a deeper infection, like necrotizing fasciitis, or they need sub sexually consultation, these are probably reasons to admit the patient to the hospital.

Now, some facilities have a short stay unit, like in their emergency department. So, if you don’t meet inpatient admission criteria, you’re likely to improve within 28 hours. Maybe the kid failed initial outpatient treatment with 48 hours of appropriate antibiotics, and they need just a day of IV. They’ve got a rapidly expanding lesion, but it’s probably IND.

The kid has a lot of pain. They can’t tolerate oral antibiotics, or they’re less than six months of age. You know, maybe that’s a patient you observe for just 24 hours in a short stay. And fortunately, cellulitis in children under the age of 2 months of age is rare, but those kids should probably be admitted for IV antibiotics as well, and you should get a blood culture in those situations. One example would be neonatal mastitis, a skin infection of the breast tissue.

Alright, so let’s talk about antibiotics. And generally, your best choice for the majority of children is cephalexin. You’d think because MRSA is everywhere. You want to avoid first generation cephalosporins, but it’s still mostly beta hemolytic strep.

And studies, including Chen et al., showed no difference between cephalexin and clindamycin. And what about length of treatment? Well, for mild cases, five days is probably just as good as ten days. But if you have moderate or severe symptoms, a week and a half is a good idea. You’ll see a lot of places start cephalexin plus clinda, or cephalexin plus trimethoprim sulfamethoxazole.

It’s still often done out in the community. A representative randomized control trial from Palin from 2013 showed that the addition of TrimSulfa did not improve outcomes in a very large cohort that contained lots of children. So the bottom line is, even in patients where you think they might have MRSA nasal carriage?

Monotherapy with cephalexin is fine. So, if you’re doing outpatient treatment or you’re transitioning patient to oral treatment after IV, the first line therapy is oral cephalexin. The dose is 50 mg per kg per day, divided every 8 hours or 3 times a day with a max of 500 mg per dose. If the patient has a true allergy to cephalosporins, you do oral clindamycin, 10 mg per kg per dose, every 8 hours, or 3 times a day, with a max of 1, 800 mg per day, or 600 mg per dose.

If you see treatment failure, that’s another reason to do clinda. So if the kid’s not getting better in 48 to 72 hours on cephalexin, you can do the same dosing of clinda that I just mentioned a moment ago. For inpatient treatment, first line therapy is intravenous cefazolin. So 20mg per kg per dose every 8 hours with a max of 1g per dose.

Cephalosporin allergy, you would use clindamycin. And yes, PO and IV clindamycin are bioavailable. But if you’re admitting somebody, there’s probably a reason that they may not be able to take PO. So if it’s IV, it’s 10mg per kg per dose every 8 hours with a max of 900mg. So it’s higher than the max for PO.

And so again, I will reiterate that 5 days for very mild cellulitis is totally okay. But you could do 10 if that’s what you do locally. Moderate and severe, you need 10 days. Some specific scenarios that you might want to deviate from cephalexin, so if there’s a mammalian bite, some don’t need prophylactic antibiotics, and I’ll admit, There’s no randomized controlled trials of dog bites.

I don’t think the IRB would approve that, but if you are concerned about infection, amoxicillin clavulonate, 22. 5 milligram per kilogram with a max of 875 milligram per dose, oral twice daily. If the kid looks well, And the degree of redness or symptoms is very mild, you could do five days, that’s totally okay.

If the child got an infection in a seawater or freshwater environment, there’s some different organisms like Aeromonas and Vibrio and other things that you want to consider. So you would still start with Cephalexin, but you would add Ciprofloxacin, 10 mg per kg, max 500 mg per dose, twice a day. Or Trimethoprim sulfamethoxazole twice a day with an overall treatment length of 5 to 10 days.

And if you definitely think that it’s methicillin resistant staphylococcus aureus that you’re treating, mild cellulitis, you could do trimethoprim sulfamethoxazole or clindamycin. If you’re sure it is moderate MRSA cellulitis, you could do a trial of oral antibiotics with close follow up or vancomycin IV.

And if they have severe cellulitis, Vancomycin IV, and if you have Staphylococcal Scalded Skin Syndrome, consider adding clindamycin. Switch to oral antibiotics as soon as the patient looks better. And there are some cases of cellulitis where you might need a specific subspecialist. So a general surgeon should be consulted if you have cellulitis of the breast, perianal tissues, perineal tissues, a complex of recurrent pilonidal abscess, Though you could drain these and have them follow up as an outpatient.

Or the cellulitis is just large and complex, or you think it’s going to need drainage in a day or two. ENT should see invasive neck cellulitis, especially with significant symptoms, or you’re concerned that it might be a deeper infection. ophthalmology and or ENT for orbital or periorbital cellulitis, orthopedics for septic arthritis, tenosynovitis or osteomyelitis, and dental or oral maxillofacial surgery for facial cellulitis due to dental infection where the kid might need to be admitted.

All right, so that’s all for this episode on cellulitis. Remember, most cases are mild or moderate, and the child will do incredibly well with oral antibiotic therapy, which should generally be cephalexin. You do not need labs, and especially a blood culture, for the vast majority of children with infections like cellulitis or erysipelas.

A fever does not mean you have to get a blood culture or admit the kid. If they haven’t started antibiotic therapy yet, and they look better after they defer vest with antipyretics, Start oral antibiotic therapy and develop a close follow up plan. Well, I hope this episode is useful. Ultimately, my goal is to deliver succinct evidence based information to help you on your next shift in the ED.

If there’s other topics that you want to hear about, send them my way. I’ll take an email. I’ll take a comment on the blog. I’ll take a direct message on Twitter or X or whatever it’s called. Leave a review on your favorite podcast site. That helps other people find it. And if more people can learn, I’m going to be happy about it.

Encourage your colleagues to listen and subscribe to the podcast. And thank you so much for your time and attention. I know you’re all busy out there. For PEM Currents, the Pediatric Emergency Medicine Podcast, this has been Brad Sobolewski. See you next time.

Laryngomalacia, is the most common cause of infant stridor. Early diagnosis is crucial as it can impact a child’s growth and development. Most infants get better on their own, but those with severe symptoms need surgical interventions like supraglottoplasty. Learn all about diagnosis and management of this common problem in this brief podcast episode.

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References

Klinginsmith M, Goldman J. Laryngomalacia. [Updated 2022 Jun 5]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK544266/

Hartl TT, Chadha NK. A systematic review of laryngomalacia and acid reflux. Otolaryngol Head Neck Surg. 2012 Oct;147(4):619-26.

Boogaard R, Huijsmans SH, Pijnenburg MW, Tiddens HA, de Jongste JC, Merkus PJ. Tracheomalacia and bronchomalacia in children: incidence and patient characteristics. Chest. 2005 Nov;128(5):3391-7.

Isaac A, Zhang H, Soon SR, Campbell S, El-Hakim H. A systematic review of the evidence on spontaneous resolution of laryngomalacia and its symptoms. Int J Pediatr Otorhinolaryngol. 2016 Apr;83:78-83.

Transcript

Note: This transcript was partially completed with the use of the Descript AI

Welcome to another episode of PEMCurrents, the pediatric emergency medicine podcast. As always, I’m your host, Brad Sobelewski. Your time is valuable and was mine, and that’s why I release these brief episodes, focus on a single topic, get you in, get you out, teach you something. Today, let’s talk about The most common cause of noisy breathing in newborns and infants, laryngomalacia. You’ve all seen this, or should I say you’ve all heard this, And you will hear the symptoms of stridor and noisy breathing, often positional, and it can impact growth and development.

Now I always thought it was just because airways are small and they’re floppier and therefore noisier, but All infants have small airways and all of their cartilage is soft, so there’s more to the picture. So why does this happen? Well, it could be neurologic function. You could have abnormal tone of the laryngeal nerve. You might have an imbalance of demand supply during inhalation in some infants.

And reflux isn’t a direct cause, but approximately sixty percent of infants with laryngomalacia do also have gastroesophageal acid reflux disease. And we know that reflux can irritate and swell the upper airway, potentially worsening obstructive symptoms. So the incidence is unknown, but it’s probably about one in two to three thousand infants. But it might underestimate it because lots of mild cases don’t actually present clinically, and they’re not diagnosed endoscopically. In the past, we thought there was a male predominance, But it’s equally common in female infants, and black and Hispanic infants may have a higher risk compared with white infants.

Low birth weight has also been suggested to be a contributing factor. So when you’re looking at an infant with possible laryngomalacia, You need a very detailed birth history. So were there any surgical procedures or intubations? Parents need to give you information about breathing difficulties, Especially, noisy breathing or episodes of apnea, noisy breathing that worsens with feeding or while lying down may indicate laryngomalacia. You should also ask detailed questions about feeding habits, weight gain, or if the child’s experiencing failure to thrive.

There are, of course, other causes of noisy breathing in infants that are on the differential. Unilateral vocal cord paralysis Often happens after surgery. So if the kid’s not had surgery, it’s pretty much less likely. Bilateral vocal cord paralysis, which is twice as worse, Prevents with biphasic stridor and may require tracheostomy if respiratory distress is significant. Laryngeal papillomatosis can cause a hoarse cry in upper airway obstruction, and it also appears in infancy.

Subglottic hemangiomas are fortunately rare, but they can cause Torrey Strider. Hemangiomas distributed like a beard are suggestive of this. Subglottic stenosis happens via scarring in the subglottic region due to a previous intubation. So stridor may be present in these infants, but it doesn’t typically change with position. And the child’s history would include a NICU stay and an intubation.

Tracheomalacia and bronchomalacia can coexist with a ringomalacia. Expiratory airway sounds are commonly heard in addition to the stridor, and diagnosis needs scope. We’ll talk more about those in a moment. Vascular ring is a rare cause of airway obstruction in infants, and you’d have feeding difficulties in stridor. The best way to diagnose this is through imaging like CT scans.

And finally, you should always think about foreign body aspiration Even in a child who is not eating solids yet. Remember, an older sibling could feed them something. I have seen this before. So an infant is in respiratory distress after being accompanied or the symptoms are persistent, you should always think about a foreign body, especially in the acute setting. The physical exam of a Child with possible laryngeal malaysia focuses on the airway and breathing naturally.

Make sure that the nasal passages are patent. You don’t wanna miss The oral cavity should be explored for cleft lip, cleft palate, glossoptosis, Pierre Robin sequence, or micronathia. Obviously, all of those facial and oral abnormalities can contribute to breathing and feeding difficulties. You wanna check out the neck for masses or vascular lesions. And many infants have hemangiomas, but I mentioned this before.

If you see them in a beard like distribution, worry about airway hemangiomas. So if you have an infant that has some intermittent, noisy breathing on inspiration, and sometimes it’s worse with feeding and when they lay down, you should really think about laryngomalacia. And if the infant is thriving and growing well, not having any apnea or color change, you can kinda wait it out. But if you wanna make the diagnosis definitively, you will need to have an otolaryngologist perform an awake, Flexible fiber optic laryngoscopy. That’s the primary diagnostic tool for infant stridor in general.

You get a comprehensive view of the oropharynx, the supraglottis, the glottis, the subglottic area, and the hypopharynx. For infants with laryngomalacia on scope will have some specific anatomical abnormalities. They’ll have shortened aryepiglottic folds, An omega shaped epiglottis and or redundant arytenoid tissue. And again, The awake flexible fiber optic laryngoscope is the gold standard for diagnosing laryngomalacia. The Kid is awake and breathing, and that allows you to assess the dynamic collapse of the supraglatic airway during respiration.

In the child with more severe symptoms or you’re worried about tracheomalacia, direct laryngoscopy including a diagnostic bronchoscopy in the OR under general anesthesia may provide a more comprehensive look at the Airway and the upper aerodigestive tract. So you’ll scope down to the main stem bronchi in this standpoint. Sometimes you get A triple scope, which is flexible laryngoscopy, bronchoscopy, and upper endoscopy. So three endoscopists together. I don’t know if there’s a discount.

And this is important when there’s severe symptoms or you have concern about synchronous airway lesions. Direct laryngoscopy can also be a prelude to surgical intervention if you need it. Speech therapists could be particularly valuable If there’s issues with swallowing or feeding, if you’re concerned about aspiration or swallowing deficits sometimes they’ll do a modified barium swallow study. Sleep studies can be particularly helpful to look at symptoms of obstructive sleep apnea, especially in older symptomatic infants. As I’ve alluded to before, fortunately, most children resolve on their own by about twelve to eighteen months of age.

So this management is conservative, includes upright feeding, antireflux therapy, so alterations to how you Feed, burp, and position. And no study has yet shown that proton pump inhibitors improve laryngomalacia symptoms, but they’re still widely used. What’s important to remember is that the majority of infants don’t need any intervention. However, there is a small proportion of infants that do have more severe symptoms, like poor weight gain, Feeding difficulties, obstructive sleep apnea, severe breathing symptoms, they’ll need a procedure called supraglottoplasty. And so how is that done?

Well, they could use lasers or cold steel or microdebridement, and it’s really tailored to the individual patient’s anatomy. So you could divide shortened area epiglottic folds. You can remove some redundant arytenoid mucosal tissue. You could do an epiglottopexy or a combination of some of these. And you really obviously don’t wanna scar the infant’s airway or cause glottic stenosis.

Supraglottoplasty is highly effective. Ninety five percent do great and see measurable and significant improvement in their laryngomalacia symptoms. Only about five percent need some sort of provision, And a higher risk of that is seen in patients that were under two months of age when they got the initial procedure or have neurologic or cardiac comorbidities. Neurologic comorbidities have the highest rate of revision surgery. Maybe up to three out of five patients will need a trach due to ongoing airway obstruction.

Aspiration after supraglottoplasty is the main risk, but it’s rare and not significantly associated with the procedure Self. And let’s not forget the main reason that most of you have heard of larynga Malaysia. It’s because a parent was worried about it. It can be scary to have a baby who is breathing noisily. The fear in the back of your head is that the baby could stop breathing and die.

It’s real. I’ve heard it personally. Ultimately, providing reassurance, guidance, close follow-up, and helping the parent understand what they’re doing well can go a long way into making sure that you’re not missing anything else and you’re appropriately monitoring the infant with potential laryngomalacia. Refer to ENT if you’re unsure of the diagnosis or if you feel that it will benefit the patient. It may seem a little barbaric, but an awake Bedside fiber optic scope isn’t actually that big of a deal.

Alright. So here’s some summary points. The majority of patients with Laryngomalacia will outgrow it by twelve to eighteen months. Diagnosis can be suspected clinically, but awake fiber optic Flexible laryngoscopy is definitive. Severe symptoms should be monitored and include recurrent cyanosis or respiratory distress, Apnea and failure to thrive.

These need further workup, including many times direct laryngoscopy and bronchoscopy to rule out other airway abnormalities. Surgery is generally only indicated in patients with severe laryngomalacia, and it’s called Supraglottoplasty. It’s generally well tolerated to highly effective. Patients with neurologic comorbidities or congenital cardiac disease and those less than two months of age are more likely to require revision surgery for relief of ongoing symptoms. Alright.

So that’s it for this episode. Hopefully, you learned something new about laryngomalacia. If you have any other suggestions for topics, send them my way. I’ll take emails, comments on the blog, direct messages on x or Twitter, threads, Facebook, whatever channels you wanna use. My goal is to make sure that you are ready for your next ED shift with up to date information that’s evidence based and ready to deliver to patients and families.

If you have the time, leave a review on your favorite podcast site. That helps with discovery so that more people can listen and learn. And share this episode in the show with your colleagues. I’d love to hear what they think as well. For PEM currents, the pediatric emergency medicine podcast, this has been Brad Sobolewski.

See you next time.

Meckel diverticulum is a congenital anomaly of the small intestine that can present with various clinical manifestations, including rectal bleeding and obstruction. Recognizing the characteristic features and understanding the differential diagnosis is crucial in managing patients with lower gastrointestinal bleeding. This episode will help you recognize and diagnose this surgical condition that you probably remember […]