Honestly, one of the best aspects of writing the Ped EM Morsels weekly is that I benefit from learning (or re-learning) something each week. This week is a great example of that phenomenon. We do not often have to care for the pediatric cardiac arrest, but when we do we take comfort in our advanced training and can rely on some of the basic concepts and recommendations (AHA Resuscitation Guidelines). What happens, though, when our advanced life support training steers us in the wrong direction? Let us consider the unusual case of Catecholaminergic Polymorphic Ventricular Tachycardia.
Catecholaminergic Polymorphic VTach: Basics
- Rare, but important cause of syncope or sudden death in kids.
- Mortality as high as 35% by age 30 years when untreated.
- Worse prognosis for those who present earlier in life.
- Malignant arrhythmogenic disorder that is characterized by:
- Structurally normal heart,
- Unlike Hypertrophic Cardiomyopathy.
- Unlike Arrhythmogenic Right Ventricular Dysplasia
- Normal baseline ECG (some may have borderline long QTc),
- Unlike Long QT Syndrome
- Unlike Short QT Syndrome
- Unlike Brugada Syndrome
- Unlike Wold-Parkinson-White Syndrome
- Polymorphic VTach induced by:
- Exercise or
- Emotional Stress or
- Other cause of Increased Catecholamines (ex, illicit substances)
- Generally presents between ages of 7 and 9 years.
- Uncommon to present before age 2 years.
Catecholaminergic Polymorphic VTach: Genetics
- Family history of exercise-related syncope, seizure, or sudden death is reported in 30% of cases.
- Caused by gene mutations.
- Likely several genes involved, but two are known (RyR2 and CASQ2)
- Both encode of proteins that regulate intracellular calcium!
- It is recommended that other family members are tested and treated if found to have the genes.
Catecholaminergic Polymorphic VTach: Presentation
- Typically will present with syncope and/or “seizure.”
- Often initially misdiagnosed as either seizure or benign cause of syncope.
- Mean delay in correct diagnosis is ~ 2 years.
- Resting ECG is normal.
- There is progressive ventricular ectopy as the heart rate increases.
- Between 100-120 beats per minute there is increasing complexity.
- First isolated PVCs evolve to bigeminy and then runs of nonsustained VTach.
- Eventually, sustained VTach and Polymorphic VTach are observed.
Catecholaminergic Polymorphic VTach: Daily Therapy
- It is recommended that ALL phenotypically and genotypically diagnosed individuals receive therapy.
- 1st line
- Nadalol is often used
- Implantable Cardioverter Defibrillator (ICD)
- Used for those who fail medications or for those who present with an aborted cardiac arrest.
- Should be used concurrently with beta-blockers to avoid electrical storm caused by the adrenergic surge from the shocks.
- Left Cardiac Sympathetic Denervation
- Also recommended that they avoid competitive sports or other stimulants.
Catecholaminergic Polymorphic VTach: ACUTE Therapy
- Managing a patient with CPVT who is in VTach can be tricky.
- The most important step is recognizing that the patient has Catecholaminergic Polymorphic VTach.
- Family history is integral!
- Previous history of syncope or seizure can raise suspicion for it as well.
- Standard use of Epinephrine during the resuscitation can be counterproductive.
- IV beta-blockers is considered first choice, similar to VTach storm of other etiologies.
- Given that it is Polymorphic VTach, it would also be reasonable to administer Magnesium, but this would be aimed at possible Prolonged QTc.
Moral of the Morsel
- This represents a significant challenge for us in the Emergency Department. It is rare and can present in a benign fashion (syncope). Even those who are thorough and obtain an ECG may be inappropriately reassured by the normal ECG.
- The patient who has had a SYNCOPAL event deserves consideration for multiple entities (ex, Prolonged QTc, SVT) and should have a screening ECG.
- Ask about family history! You can’t outrun your genes.
- Ask about prior syncopal events that were associated with exciting or stressful situations.
- Don’t always assume wellness just because the patient is young.
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Discussion of bleeding and bleeding disorders always garners attention. We have recently covered hemophilia, ITP, and hemorrhagic disease of the newborn, but let us turn our attention to the most prevalent bleeding disorder: Von Willebrand Disease. Certainly, making the diagnosis of von Willebrand disease is not necessarily a common goal in the ED, but knowing how to deal with it is!
Von Willebrand Disease Basics
- Inherited bleeding diathesis.
- Estimated to affect as much as 1% to 2% of the population (adults and kids).
- Caused by either too little von Willebrand Factor or von Willebrand factor that is defective.
- The function of Von Willebrand factor is to help:
- Bind platelets to injured endothelium
- Stabilize factor VIII.
- Von Willebrand is generated in the endothelial cells.
Von Willebrand Disease Types
- TYPE 1
- Partial Deficiency of von Willebrand Factor
- Most common type – >70% of cases.
- Autosomal DOMINANT
- Usually leads to MILD BLEEDING
- TYPE 2
- Defective von Willebrand Factor
- Has 4 subtypes, depending upon the specific defect.
- May be autosomal Recessive or Dominant.
- Severity varies between the subtypes, but more severe than Type 1.
- TYPE 3
- Complete Deficiency of von Willebrand Factor.
- Autosomal RECESSIVE.
- More Serious presentations.
Von Willebrand Disease Presentations
- Most often presents with mucocutaneous bleeding.
- Commonly seen:
- Easy Bruising
- Type 3 may present with serious bleeds:
Von Willebrand Disease Diagnosis
- When you are considering a new diagnosis of a bleeding disorder, it is important ask:
- Age of onset – often presents early, but may be missed until later in life.
- Location of bleeding
- Spontaneous bleeding?
- Family history – obviously… you can’t outrun your genes.
- Hemostatic Challenges (trauma or surgery) – a major inherited bleeding D/O is unlikely in a patient who has had an uneventful surgery in the past.
- There are bleeding scoring systems to help determine likelihood of bleeding disorders… but they are not validated in kids.
- Initial Work-Up
- Coagulation Studies
- Von Willebrand Specific Studies
- vWF Antigen
- vWF activity
- Factor VIII level
- Unfortunately, normal tests do not rule out the diagnosis completely.
- Involving a hematologist is necessary.
Von Willebrand Disease Treatments
- Desmospressin (DDAVP)
- Synthetic analog of vasopressin
- Promotes release of vWF from endothelial cells.
- Patients with Type 1 often respond well, but type 2 and 3 often do not.
- Can be given SubQ or IntraNasal, but IV is more effective.
- Complications = hyponatremia, facial flushing, headache, tachyphlaxis and hypo/hypertension.
- Plasma-derived vWF and Factor VIII
- Goal is to achieve vWF:RCo > 30% for minor bleeds and >50% for major bleeds.
- Loading doses:
- Minor Bleed – 30-50 IU/kg
- Major Bleed – 40-60 IU/kg
- Contains vWF and FVIII)
Use only when vWF concentrate is not available.
1 bag/6 kg body weight.
- Aminocaproic acid
- 100-200 mg/kg (max of 10 grams)
- Followed by 50-100 mg/kg/dose (max of 5 grams) q 6 hrs.
- Tranexamic acid
- 25-50 mg/kg q 6-8 hrs.
- Absorbed from the buccal mucosa and secreted in saliva!
- Can treat oral bleeding with a mouth-wash of TXA!
- Crush tablets in 10-15ml of water and have patient hold that in their mouth for 5 min before swallowing.
- Has longer half-life, more potency, and lower toxicity than Aminocaproic acid and is preferred.
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The ART of medicine is much more difficult to learn than the science of medicine; however, it is through becoming comfortable with the “grey areas” that we often experience our greatest ability to help others. It is also what prevents robots from taking our jobs.
One “grey area” that we constantly explore is that of antibiotic administration. Now, this would seem like a very well defined area and not one of nuance – if there is a bacterial infection, give antibiotics; if there is not, do not. Unfortunately, we know that it is not that clear cut. The treatment of Acute Otitis Media, which can be complicated by Mastoiditis, is a good example of the need to do complex Pro-Con equations to determine the best plan for a specific patient. While there may be purulent material present, exposing the patient to antibiotics is not necessarily the best plan always (see Serum Sickness). Another excellent example of this is sinusitis in children.
Acute Bacterial Sinusitis “Diagnosis”
- In 2013, the clinical diagnosis of Acute Bacterial Sinusitis in children was revised.
- Admittedly, the research pertaining to Sinusitis in children was often undermined by varied definitions of sinusitis, so this is hopefully helpful.
- Acute Bacterial Sinusitis can be clinically diagnosed when a child who has an acute upper respiratory tract infection (URI) presents with:
- PERSISTENT illness - any nasal drainage, or DAYtime cough, or both lasting > 10 days WITHOUT IMPROVEMENT; or
- WORSENING course – worsening or new onset of nasal discharge, DAYtime cough, or new fever; or
- SEVERE onset AND PURULENT Nasal drainage – concurrent fever > 102.1F (>38.9C) AND purulent rhinorrhea for at least 3 consecutive days.
Key Words of Sinusitis
- Just to be clear… there are some keywords that cannot be overlooked when considering this diagnosis:
- PERSISTENT and NOT IMPROVING
- These are the only tools that we have to help us make this diagnosis. More importantly, these are the words that can help us not over-diagnose it!
- There is NO lab test.
- There is NO radiographic test.
- There is NO reliable physical exam finding.
Typical Time Course of an URI
I understand; there is nothing exciting about talking about a URI. It is common. It is simple. It can be accurately diagnosed by grandparents and managed effectively by them. So why do I torture you, an experienced clinician, with this? Because, it is what makes the above make some sense.
- A URI is typically characterized by nasal symptoms (congestion and/or discharge).
- Most often the nasal discharge starts as clear and watery.
- Over time, the discharge may become thicker and more mucoid or even purulent for several days.
- As the URI resolves, the nasal drainage usually becomes less mucoid and clears.
- Fever tends to occur early in the illness.
- Often fever occurs before respiratory symptoms are prominent.
- Usually resolves in the first 1-2 days.
- The total duration is often 5-7 days.
The Tricky Part
- Kids love to collect different URIs sequentially!
- Parents will not necessarily perceive that two separate viral illnesses have occurred… they just know that their child has been sick for “2 weeks straight.”
My General Approach
- Ok, take this for what it is…
- Start off with the knowledge that only ~6-7% of children will meet criteria for Sinusitis.
- Know that the benefit of antibiotic therapy in treating sinusitis only seems to be apparent in those more severe symptoms and when the more strict criteria are applied.
- Know that the benefit of antibiotic therapy in preventing suppurative complications (orbital cellulitis or intracranial abscess) is unproven.
- Focus on the history.
- Spend a little more effort in helping the family sort out if this “2 weeks” of illness is really two separate URIs.
- Consider the time course and the onset of fever. Fever that occurs after illness has started is not typical for an URI.
- Purulent rhinorrhea occurring during the onset of fever is also odd for an URI. 3 days of purulent snot and high fevers should make you think about sinusitis.
- Have the parents help you!
- Shared decision making can be very powerful… especially with these grey areas of medicine.
- Discuss the potential for sinusitis, but how there is no definitive way to diagnose it.
- Discuss how antibiotics may help some, kids, but can also cause harm (ex, diarrhea, allergy, toxic epidermal necrolysis, etc).
- A period of OBSERVATION for those with persistent symptoms can be very helpful in sorting out the ambiguous cases.
- Don’t just throw antibiotics at all kids with “persistent symptoms” please!! They all do not have sinusitis.
Treatment for Sinusitis
- Sinusitis defined by Persistent Symptoms – antibiotics OR Observation period for 3 days.
- Sinusitis defined by Worsening Symptoms or Severe and Acute symptoms – antibiotics.
- Antibiotic Options
- 1st line = Amoxicillin
- 45mg/kg/day divided BID is sufficient if you have low antimicrobial resistance.
- 90mg/kg/day divided BID (max of 2 grams per dose) in areas with abx resistance.
- Augmentin (80-90mg/kg/day of the amoxicillin component) can be used in those with moderate or severe illness or in those < 2 years of age.
- Ceftriaxone 50mg/kg IM dose x 1
- If clinical improvement is seen at 24 hours, then transition to Oral abx.
- If clinical improvement is not seen at 24 hours, then parental abx may be required.
- Penicillin allergic?
- Consider cefdinir, cefuroxime, or cefpodoxime.
Wald ER1, Applegate KE, Bordley C, Darrow DH, Glode MP, Marcy SM, Nelson CE, Rosenfeld RM, Shaikh N, Smith MJ, Williams PV, Weinberg ST; American Academy of Pediatrics. Clinical practice guideline for the diagnosis and management of acute bacterial sinusitis in children aged 1 to 18 years.
Pediatrics. 2013 Jul;132(1):e262-80. PMID: 23796742
. [PubMed] [Read by QxMD]
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It is easy to fall into a potentially callous mindset in the Emergency Department – “If it isn’t an emergency, then it can wait to see the Primary.” While that may be a valid statement, it won’t win you any bed-side manner awards. It also mandates the ability to determine what presentations are not due to underlying emergent issues, which can be tricky in kids (hence the common theme of remaining vigilant).
Additionally, if the family brings their kid at 3am, they likely think it is at least important enough to warrant your potential concern. Fortunately, many times the “important” issue revolves around a typical oddity of being a young child (like periodic breathing). Being comfortable with some minor issues, particularly neonatal issues (currently, there are 26 categorized neonatal Morsels), can make you more comfortable with dealing with the potential emergent conditions that exist within seemingly innocuous presentations. Umbilical Granuloma is a good example.
Umbilical Cord Basics
- In utero, the umbilical cord is one of the most important structures. After birth, it becomes useless.
- All of the structures associated with it should be obliterated or recede.
- When they don’t, problems can occur.
- Umbilical Cord Stump may remain attached from 3 to 45 days.
- Mean duration was ~2 weeks.
- Delayed cord separation is associated with some immune problems.
- The care for the umbilical stump vary from institution to institution.
- Some recommend no care.
- Some recommend soaps or alcohol application.
- All fear umbilical stump infection (omphalitis)!
- Most common cause of Umbilical Masses.
- Form within the 1st week after cord separation.
- are moist and pink
- range in size from 1 mm to 10 mm.
- can be associated with some sanguinous or even greenish discharge.
- Most often treated, successfully, with Silver Nitrate application.
- Silver nitrate is not without its complications.
- Some advocate for application of salt crystals instead.
- Silver Nitrate can act as an antiseptic, an astringent, or a caustic agent (depends on the concentration)
- While it can help resolve an Umbilical Granuloma, it can also burn the tissue around it!
- Contact with normal tissue should be avoided.
- The Umbilical Granuloma should be dried before application to limit the potential spread of the silver nitrate by the discharge from the Umbilical Granuloma.
- When dealing with a persistent Umbilical Granuloma:
- don’t just keep adding silver nitrate.
- If the Umbilical Granuloma persists after 2 applications, consider other potential issues.
Other Umbilical Cord Issues
- The issue we all worry about.
- Can complicate the other issues as well.
- This is an emergency!
- Omphalomesenteric Remnants
- The Omphalomesnteric Duct (OMD), also known as the vitelline duct, is present in early gestation.
- The OMD connects the yolk sac with the developing GI tract.
- It should involute at week 8 or 9 of gestation.
- Remnants occur in ~2% of the population.
- May persist as tissue attached to ileum = Meckel’s Diverticulum
- May present with painless rectal bleeding.
- Most common of these anomalies.
- May persist as a cyst beneath the umbilicus = OMD Cyst
- May persist as a patent Fistula between the GI tract an umbilicus.
- Present as persistent drainage, often with fecal material!
- May persist as a Polyp at the umbilicus = Umbilical Polyp
- Present as a Very Red mass within the umbilicus.
- Can contain gastric or intestinal tissue.
- Do not resolve with silver nitrate.
- Often confused with Umbilical Granuloma!
- Urachal Remnants
- Fistula – present with clear drainage or drainage of urine from the umbilicus.
- Cyst – present with painful mass between the suprapubic region and the umbilicus.
- Both can become infected.
What to Do?
- Look for signs of infection (obviously). If infected, do sepsis work up and consult surgery!
- If it appears as if you are dealing with a simple Umbilical Granuloma, gently and carefully apply silver nitrate and arrange follow-up.
- Refer to Surgery:
- If the Umbilical Granuloma / mass did not respond to silver nitrate.
- If it is unusually RED.
- If there is significant drainage.
- If there is a mass associated.
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We all know kids love to get objects stuck in areas where no foreign body should be. We have discussed this several times (the Nasal Foreign Body, the Ear Foreign Body, and the Ingested Foreign Body), but the Aspirated Foreign Body is the one that gets our attention the quickest. That is, unless, we missed it at first. Unfortunately, a foreign body in the bronchus may be deceptive and present in a delayed fashion… so, we must be vigilant (as always).
Young Kids are at Risk
- It would seem intuitive that younger kids are at greater risk for aspiration… but why?
- Young kids:
- put random things in their mouths. They like to explore their world… and don’t know what is not edible (hmmm crayons).
- don’t chew their food well. They do not have molar teeth and are prone to have insufficient mastication.
- are easily distracted. It is not a great idea to yell at your sister while stuffing hotdogs in your mouth, but they don’t know that yet.
- don’t protect themselves well. They have relatively high epiglottis and immature swallowing coordination.
Drama is Easy to See, but Subtle can be Dangerous too.
- The aspirated foreign body that leads to respiratory distress won’t be missed by many.
- Unfortunately, an aspirated foreign body may go undiagnosed and this can lead to significant sequelae.
- Persistent febrile illness
- Chronic cough
- Recurrent pneumonia
- Recurrent / persistent “croup”
- Poorly controlled “asthma”
- Lung abscess
- Progressive respiratory distress
- Delayed diagnosis of an aspirated foreign body is associated with increased incidence of complications.
- This is particularly true for any vegetable matter (ex, peanuts), which cause more inflammatory response the longer it is in the body.
Factors Related to Delayed Diagnosis
- Kids 3 years of age and younger are more likely to be diagnosed with foreign body in a delayed fashion.
- These kids are less able to give a reliable history and more at risk (as mentioned above) for aspiration.
Negative Chest X-rays
- Just because the child’s CXR is negative, doesn’t mean that there is not a foreign body!
- It just means your job is more difficult.
- ~50% of known cases of aspiration will have normal chest x-rays.
- Special projection films (decubitus position, exhalation films) can help, but should not be used to completely rule out the condition.
- Normal X-rays should not preclude bronchoscopy in a patient that you have concern for an aspirated foreign body.
- That is taken from the ENT literature — in case you need some support when dealing with consultants.
Care provider negligence
- When no one is supervising, badness can happen (says Captain Obvious).
- One study showed that having No history of a witnessed aspiration episode was associated with delayed diagnosis.
- A lack of a parental report of an aspiration event should not sway you from considering retained foreign body in a child who’s clinical picture suggests it.
Lack of typical symptoms
- With a radio-opaque foreign body, the diagnosis will depend on the history (hopefully someone saw something) and the physical exam.
- Unfortunately, after the initial choking event, the aspirated foreign body may cause little if any symptoms, particularly if it falls to the bronchus.
- The diagnosis is then delayed until symptoms of complications arise.
- Location matters:
- Larynx – typically will have symptoms of obstruction, dysphonia, or hoarseness. If blockage is complete, then can have cyanosis and severe distress / arrest.
- Trachea – similar to larynx in presentation, but can have biphasic stridor, dry cough. Often appear uncomfortable or scared.
- Bronchus – 80-90% of foreign bodies are found in the bronchus. Only 65% will have triad of cough, wheeze, and decreased breath sounds! Can be asymptomatic!
- The absence of symptoms and signs does not rule out the possibility of foreign body if the child has a concerning history of aspiration.
We were not vigilant
- Simply put, we didn’t think about the possibility.
- The diagnosis can be rather difficult, as it may present similar to other conditions:
- Recurrent Croup and Croup are commonly diagnosed instead of Foreign Body.
- Asthma, bronchitis, pneumonia, laryngitis, and URI are other common mis-diagnoses.
Believe the Parents
- Multiple studies have shown that, while a variety of clinical signs may be seen with an aspirated foreign body…
- The most sensitive clinical indicator for an aspiration is there being a “Witnessed Aspiration Episode.”
- Since the physical exam and radiographs may be unenlightening, the parental report of a choking crisis needs to be taken seriously!
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Without question, kids will put the oddest objects into their orifices… and then say that they don’t know how they got there. While this can lead to same comic relief to your otherwise stressful shift, managing a foreign body can also generate its own level of stress. We have great hopes for the offending foreign body being easy to remove as we walk into the room, but then can be derailed by a tenacious monster that refuses to leave and a wailing and flailing child. Those don’t make for any comic relief.
We have discussed many topics with respect to the pediatric foreign body (Aspirated FB, Nasal FB, Button Batteries in the nose, Esophageal Button Batteries), but let us look at the other common entity – the Ear Foreign Body.
- The anatomy of the ear seems simple… an external portion, a tube, and the ear drum. Naturally, it is more complicated than that.
- The External Auditory Meatus can be divided into two portions:
- The lateral third – cartilaginous portion.
- The medial two thirds
- More narrow than the lateral third.
- Lined with a very vascular and highly sensitive thin layer of skin.
- Prone to bleeding with even slight trauma.
- Naturally, the position of the patient can affect your ability to successfully remove the foreign body.
- Make sure that the patient is in a comfortable position for him/her.
- Sitting on a parent’s lap, sideways with the ear in question easily visible will often be the first position of choice.
- This will allow the patient’s legs to be between the parent’s and offer security.
- Also allows for the torso to be supported and arms kept safely out of the way.
- Teaching the family member how to do this can save everyone some time and sweat.
- Equally important is to make sure that the patient is in a position that allows you to be comfortable.
- You may be very good a yoga, but there is no need to demonstrate your skills while attempting to remove a foreign body.
- Anticipate that this will not be easy and may take a few minutes… so position your tools within easy reach.
- The position of the Foreign Body is also very important.
- Foreign bodies that are in the medial two thirds of the canal are much more problematic and more difficult to remove.
- In this area, the patient will be more uncomfortable and less likely to hold still.
- You are also more apt to cause trauma (to the canal or TB) with foreign bodies that are in this region.
- You need to have optimal circumstances and be very careful with foreign bodies in the medial two thirds of the canal.
Pain Management Matters
- Even the most cooperative child will loose the ability to calmly sit still as you scrap and claw at a foreign body, especially one in the medial two thirds of the canal.
- Be kind. Use some pain medications when appropriate.
- A common trick is to use topic lidocaine.
- It is important to first make sure that there is no perforation of the TM before pouring any fluids into the canal.
- This is very helpful with insects that are entrapped, as it will drown the insect and make everyone’s job easier.
- Ketamine is great too!
- Ok, so this isn’t something to pull out right away, but occasionally, the child with an ear foreign body will need procedural sedation.
- Short acting agents would be ideal (propofol). ENT would likely go to the OR for anesthetic gas.
The Object Matters
- A endless variety of foreign bodies have been removed from ears (from cotton to cheese; from bead to popcorn kernel; from eraser to putty).
- There are characteristics that can make the Foreign Body more difficult to deal with:
- Vegetable Matter (food, beans, etc)
- Do not use irrigation as this may cause the foreign body to swell and become more entrapped.
- Button batteries
- Cause liquefaction necrosis and need to be removed promptly.
- Sharp objects
- If not removed very carefully, may cause more injury and damage during the removal.
- Smooth, round objects
- Difficult to grasp.
- Especially challenging when they are in the medial 2/3 of the canal.
The Tool Matters
- Having a wide array of tools can help you adapt to the various challenges that each foreign body offers.
- These can come in handy:
- Forceps (alligator and Hartman)
- Frazier suction
- Cerumen loop
- Right-angle ball hook
- Schuknecht foreign body remover
- Aural irrigation devise
- Can be made using a 60 ml syringe and an 18 gauge angiocath.
- Ensure that the water is body temperature (so you don’t make the child vertiginous).
- Otomicroscope would be quite handy.
Knowing Your Limits Matters
- Occasionally, you won’t be successful.
- When should you refer to the ENT doctors?
- Unable to remove after multiple attempts.
- Multiple attempts increases risk of complications, which can lead to other long term issues.
- Tightly wedged objects
- Objects resting against the TM
- Sharp objects
- Button batteries
- These, however, cannot wait until the next day in the ENT office.
- If you can’t get it out, then the ENT needs to come in to get it out.
- After you have successfully removed the Foreign Body, your job is not done.
- Ensure that all parts have been removed.
- Especially important for insects.
- Residual barbed insect legs can lead to inflammation and damage.
- Ensure that no other orifice has a Foreign Body.
- Kids are tricky.
- Check their other ear and the nostrils!!
- Important to educate the patient and family about the hazard of the foreign body (this time it was the ear, next time it might be the airway).
- If there was some trauma to the canal (laceration, inflammation), then prescribe antibiotic otic drops and give water precautions. Follow-up examination will be important.
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