The best part of working in the Emergency Department at Carolinas Medical Center is being surrounded by so many brilliant colleagues. I learn something new every shift (and, in truth, is where the majority of these Morsels are baked). Today’s Morsel is a reflection of that fact.
We evaluate headache often. With the mantra of “worst first” echoing in our ears we naturally consider meningitis, but, fortunately, we are often able to exclude it based on our exam. In previous Morsels, we have discussed concerning characteristics of headaches as well as some interesting causes of headaches (AVM, meningococcemia, pseudotumor). What if, however, the patient has had viral meningitis previously? What is Mollaret’s Meningitis anyway?
Can be separated into two main categories:
Recurrent Bacterial Meningitis
- Bacterial meningitis untreated is almost always fatal.
- Recurrence implies that it was treated, but there is an underlying cause for the bacteria to gain access to the CSF again. Examples:
- Congenital middle ear abnormalities
- Persistent dermal sinus along the spinal column
- Basilar skull fractures
- Sinusitis, Mastoiditis, Deep Space Abscess
Recurrent Non-purulent Meningitis
- Recurrent non-purulent meningitis can be due to a wide variety of entities including infectious as well as non-infectious causes.
- Bacteria, spirochetes, fungi, protozoa, viruses.
- Tumors (ex, epidermoid cysts and craniopharyngioma)
- Inflammatory Conditions (ex, Sacroid, Systemic Lupus Erythematosus)
- Drugs (drugs can always cause badness)
- In 1944, Pierre Mollaret first described Recurrent Benign Lymphocytic Meningits due to HSV-2.
- Recurrent (at least 3 episodes), benign and brief (usually 2-5 days) episodes of aseptic lymphocytic meningitis.
- Lymphocytes have characteristic appearances:
- Large activated monocytes with several deep clefts in the nuceli called:
- “Cloverleaf nucleus,” “Footprint-shaped nucleus,” “bean-shaped nucleus.”
- Also referred to as “Mollaret’s cells.”
- These cells are most likely present in the first 24 hours of the illness.
- Onset of disease has been observed from 5 years to 83 years of age. Mean is 35 years of age.
Mollaret’s Meningitis Causes
- Most cases are due to Herpes Simplex Virus type 2 (HSV-2).
- HSV-1 and Human herpesvirus 6 (HHV-6) have also been associated.
- It is a good idea to ask about recent herpes lesion outbreaks in your patients with headaches.
Mollaret’s Meningitis Treatment
- This is a benign and self-limited disease, so does not officially require therapy.
- Intravenous acyclovir is often used initially, while diagnosis is being clarified.
- 10 mg/kg three times a day for 7-10 days.
- Intermittent or continuous prophylaxis has also been used with the goal of preventing the recurrence.
Poulikakos PJ1, Sergi EE, Margaritis AS, Kioumourtzis AG, Kanellopoulos GD, Mallios PK, Dimitrakis DJ, Poulikakos DJ, Aspiotis AA, Deliousis AD, Flevaris CP, Zacharof AK. A case of recurrent benign lymphocytic (Mollaret’s) meningitis and review of the literature.
J Infect Public Health. 2010 Dec;3(4):192-5. PMID: 21126724
. [PubMed] [Read by QxMD]
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There are many conditions that exist in both Pediatric and Adult patients who present to the ED. Some of them require special consideration when you are evaluating the special population of pediatric patients (please don’t say “kids aren’t little adults”… that drives me crazy). For instance, DKA management often requires different considerations in children. Another entity that requires some additional considerations is Facial Nerve Palsy.
Facial Nerve Palsy in Kids – Basics:
- Facial Nerve Palsy is commonly encountered throughout one’s lifetime, but is relatively rare in kids.
- Less common in younger children and infants than it is in teenagers and adults.
- So don’t be cavalier when diagnosing a young child with “Bell’s Palsy.”
- Outcomes are related to underlying cause as well as severity upon presentation.
Facial Nerve Palsy Severity Grading
- The House-Brackmann Facial Nerve Grading System is used to grade the severity of the palsy.
- Grade 1 – Normal function
- Grade 2 – Mild dysfunction: normal symmetry at rest, able to completely close the eye with minimal effort.
- Grade 3 – Moderate dysfunction: noticeable but not severe synkinesis, with effort can close eye, asymmetric mouth movement with maximal effort.
- Grade 4 – Moderately severe dysfunction: obvious weakness, incomplete eye closure, asymmetric mouth movement.
- Grade 5 – Severe dysfunction: only barely perceptible motion, asymmetric at rest.
- Grade 6 – No movement.
Facial Nerve Palsy Causes to Consider in Kids
Abridged list– but the big ones to consider are:
- Idiopathic (Bell’s Palsy)
- Often reported to be the most common cause of Facial Nerve Palsy in kids (40-70% of cases).
- More recent literature demonstrate lower percentages, likely from being able to detect other potential infectious cause.
- Otitis media, mastoiditis, parotitis, meningitis
- EBV, Mycoplasma pneumoniae, HSV, VZV, tuberculosis, HIV, poliomyelitis, mumps
- Lyme Disease (the most prevalent causes in endemic areas!)
- Ramsay-Hunt Syndrome (Herpes Zoster cephalicus) – look for vesicles on TM or in mouth
- Inflammatory Conditions
- Fracture of the temporal bone
- Skull base fractures
- Perinatal trauma (compression from forceps)
- Trauma to parotid / cheek region
- Parotid tumors
- Brainstem masses
Facial Nerve Palsy Evaluation
- The evaluation is predicated upon a thorough history and physical exam, mindfully considering the above potential causes.
- Key Points to Contemplate:
- Condition is progressively worsening over 3 weeks or lacks improvement after 3 months – concerning for CNS or neoplastic process.
- Recurrent Facial Nerve Palsy – is rare, therefore warrants specialty evaluation.
- Bilateral Facial Nerve Palsy – likely due to a Lyme Disease or EBV infection or neurological cause (Guillan-Barre Syndrome).
- Aural Symptoms – Bell’s Palsy can have this, but so too can Ramsay-Hunt Syndrome, Otitis Media, Cholesteatoma, or tumors! Be thorough with your physical exam.
- Recent Immunizations - Facial Nerve Palsy has been related to rabies, polio, tetanus, and influenza vaccinations. Causation is a different story.
- Failure to Thrive, Persistent Fever – Consider occult neoplasms.
- Other Cranial Nerves Involved or Abnormalities on Neuro Exam – No longer a simple Facial Nerve Palsy… investigate for badness.
- Neuro-imaging would be recommended for:
- Any signs of Central Nervous System involvement,
- Suspect malignancy,
- Suspect trauma,
- Evidence of Middle Ear Cholesteatoma,
- Peripheral Facial Nerve Palsy progressing beyond 3 weeks,
- Peripheral Facial Nerve Palsy without improvement after 6 months,
- Recurrent Facial Nerve Palsy
- Be cautious with a patient who is younger than 2 years of age… Bell’s Palsy is less likely.
Idiopathic Facial Nerve (Bell’s) Palsy Treatment
- Eye Care!
- The most important therapy consideration!
- Artificial Tears during daytime.
- Moisturizing eye ointment (ex, Lacrilube) during sleep.
- There is adult literature that supports the use of steroids; however the literature does not contain many pediatric patients.
- In addition, the vast majority of children with Bell’s Palsy improve without therapy, so studies are often underpowered to determine a difference between those who received steroids and those who did not.
- If you elect to use steroids:
- Prednisolone 1 mg/kg (max 60mg) per day x 7 days and then taper off.
- Best to initiate within first week of onset.
- Consider other comorbidities (diabetes, hypertension, occult leukemia) before prescribing.
- Some would advocate for a screening CBC because of this.
- Has not been shown to be helpful on its own with adults or children.
- In adults, there is some evidence that it is beneficial when used with steroids.
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We have discussed numerous common neonatal issues (ex, Analgesia, Umbilical Granuloma, Neonatal Tooth, Hyperbilirubinemia). Many of us have learned that neonates are challenging and demand respect. Even the most proficient practitioner will be apprehensive of the sick neonate, given that there are so many conditions to consider with so few clinical clues to help sort through them (ex, ALTE, Omphalitis, PGE1, Neonatal Resuscitation, Hypothermia).
While severe infections, congenital anomalies, and unusual metabolic conditions need to be pondered, it seems completely unfair that you should also have to consider cancer in neonates!!
Neonatal Leukemia - because life is not fair.
Neonatal Leukemia – Basics
- Neonatal Leukemia is, fortunately, rare!
- Estimates of 1-5 per MILLION live births.
- While rare, it does occur (our group just diagnosed a neonate with a WBC >900,000).
- <1% of all childhood leukemia is diagnosed during the neonatal period.
- Neonatal leukemia is not the same as childhood leukemia.
- Neonatal leukemia is more likely to present with poor prognostic factors.
- Neonatal ALL has a disease-free survival rate of ~10% compared to >70% in older children.
- Neonatal leukemia is the leading cause of death in neonate due to neoplastic disease.
- Trisomy 21 (Down Syndrome) and 11q23 translocation are the most common chromosomal aberrations associated with neonatal leukemia.
- Timing of Presentation:
- Some show signs at birth.
- Some within a few days of birth.
- Others present between the 3rd and 6th week of life.
- This group may present in a more vague manner with failure to thrive, pallor, fever, and lethargy.
Neonatal Leukemia – Signs and Symptoms
- Present in the majority of ALL cases (85%), but only 49% of AML cases.
- WBC > 50 x 10^9/L
- Complications due to Hyperleukocytosis:
- Leukostasis Syndrome
- White Cells plug up the micro-circulation.
- Heart Failure
- Respiratory Failure (hypoxia, pulmonary infiltrates, tachypnea)
- Neurologic insults (somnolence, coma, retinal hemorrhage)
- Seen in about 80% of the cases.
- Liver is more often enlarged than the spleen.
- Other Lymphadenopathy is seen in only ~25% of cases.
- Seen in about 60% of cases.
- Caused by cutaneous leukemic infiltrates.
- A firm blue, red, or purple nodular eruption.
- “Blueberry muffin baby.” – similar to what is seen with congenital infections.
- Reported to be the INITIAL presenting sign in about 50% of cases!
- Keep this on your list of items to look for in the neonate!
- May precede the other signs by as much as 4 months!
- Bone Marrow Suppression (due to infiltrative disease)
- CNS Infiltration
- Cranial Nerve Palsies
- Bulging Fontanelle
- Intracranial Hemorrhage
- Pulmonary Hemorrhage
Neonatal Leukemia – on the Differential
- Leukemoid Reaction
- Causes elevated WBC counts and Hepatomegaly.
- May also have BlueBerry Muffin Baby appearance.
- Seen with Congenital Infections.
- TOxoplasmosis, Rubella, CMV, HErpes, Syphilis, Listeria.
- Also with Sepsis.
- Typically have Intrauterine growth retardation and/or microcephaly.
- Other Congenital Neoplasms (Neuroblastoma)
- Hemolytic Disease of the Newborn
- Congenital HIV Infection
Neonatal Leukemia – Initial Work-up
- The definitive diagnosis will not be made in the ED, but your suspicion for it should lead you to obtain:
- CBC with Differential
- Peripheral Smear
- Work up for Congenital Infections!
- During the admission, the child will have Bone Marrow Aspirate performed to help make definitive diagnosis.
Orbach D1, Sarnacki S, Brisse HJ, Gauthier-Villars M, Jarreau PH, Tsatsaris V, Baruchel A, Zerah M, Seigneur E, Peuchmaur M, Doz F. Neonatal cancer.
Lancet Oncol. 2013 Dec;14(13):e609-20. PMID: 24275134
. [PubMed] [Read by QxMD]
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On occasion, I get the honor of being to “asked” to help with my daughter’s homework. It isn’t common, fortunately, as I don’t recall middle school algebra that well, but recently I was emphasizing the fact that you cannot expect excellent results from complicated systems (equations) if you fail to do the basics correctly.
Often we get distracted with “advanced” strategies and techniques. While advanced procedures can be effective, suboptimal performance of the basics will undermine the entire project. This concept, I think, is easily reflected when we are involved in resuscitations. Focusing on the choice between lidocaine, procainamide, and amiodarone is fruitless if the team is not performing the basics optimally. Without question, The Basics are the Best!
We all know that the American Heart Association has emphasized the focus on the basics, and we recently discussed the need to de-emphasize pulse checks, but let us now look at how to optimize our chest compressions!
What are Quality Chest Compressions
- For pediatrics patients quality chest compressions require:
- Rate of 100 compressions per min
- Compression Depth of 1/3 AP diameter
- Compressions that allow for complete recoil of the chest!
- This is just as vital as the depth and the rate, but often under appreciated!
- It is during recoil phase that the heart chambers fill with blood and the coronary arteries have blood flow.
Quality Chest Compressions Matter
- Goal of delivering CPR is Return of Spontaneous Circulation (ROSC) and Maintaining Cerebral Perfusion.
- Obvious ROSC is important, but survival with good neurologic outcome is the real goal.
- Quality chest compressions can generate good MAP.
- This is integral to having a chance to provide cerebral perfusion!
- Sutton et al. showed that quality chest compressions can generate systolic BP >80 and diastolic BP >30!
- Quality chest compressions can lead to improved survival.
- When compared to suboptimal chest compressions, quality chest compressions have been associated with improved survival in the first 24 hours.
- Obviously, this does mean overall survival, but you have to start somewhere.
Optimize Chest Compressions
- Since chest compressions are so important, one of the most important roles a team leader has is to ensure they are being optimized.
- Use a BackBoard!
- Even with adults this is important, but particularly true in children.
- Often the compression of the gurney’s mattress will significantly diminish the actual AP compression of the patient!
- The backboard can be easily overlooked and if not present, all of the efforts are being undermined!!
- Get the timing down! Use a Metronome!
- Often it is recommended to do compressions to the beat of a song…
- But even if we all sing the same song, the tempo of that song can easily be misjudged.
- Take the guess work out of it… is an automatic timing system.
- Use a metronome to help define what 100/min is.
- There is “an app for that.” Some apps will use your smartphone’s flash to signal that rate… which give nice visual cues to the compressor.
- There are also “fancy” commercial products available that can give cues to the timing and depth of compressions, but they aren’t free.
- Use End-Tidal CO2 to provide feedback.
- Studies show that EtCO2 can be a surrogate maker of blood flow produced during compressions in both adult and pediatric patients.
- CPR is a low flow state, and as such, EtCO2 becomes less dependent upon CO2 production and ventilation and is more related to Cardiac Output.
- Since pediatric arrests are usually due to respiratory (or infectious) etiology, do not rely on the initial EtCO2 value.
- The value is more reliable after 1 min of compressions.
- Goal EtCO2 of between 10-15 during chest compressions.
- Can be used as a feedback device… as it indicates quality compressions are being done.
- If EtCO2 <10, ROSC is unlikely. Double check your quality characteristics!
- If EtCO2 increases substantially during chest compressions, this likely indicates ROSC.
- Using this method can help decrease interruptions in chest compressions for “pulse checks.”
- Just seeing an increase would not lead me to stopping compressions… I’d likely complete that 2 min cycle before checking for pulse.
- Mandate the Rotate!
- While we work with many true heroes, this is not a time to allow someone’s self-sacrifice to interfere with quality chest compressions.
- Even the strongest and biggest person will get tired.
- The power to do quality chest compressions is similar to that of running or swimming.
- While the compressor may be a physically fit individual, he/she will not sprint at the same rate the entire time.
- Studies show there is a measurable and significant decrement in quality of chest compressions at 2 minutes for both adult and pediatric patients!
- Interestingly, the rate and depth often stays the same, but the compressor begins to lean on the chest.
- This decreases the chest recoil!! We cannot have that.
- Do not ask if the compressor is “ok.”
- Tell him or her that it is time to rotate rapidly and have the next person prepped and ready to take over without interruption!
- Even if it is Superman, tell him it is time to rotate for the sake of optimizing your chest compressions!
Sutton RM1, French B2, Niles DE3, Donoghue A3, Topjian AA3, Nishisaki A3, Leffelman J3, Wolfe H3, Berg RA3, Nadkarni VM3, Meaney PA3. 2010 American Heart Association recommended compression depths during pediatric in-hospital resuscitations are associated with survival.
Resuscitation. 2014 Sep;85(9):1179-84. PMID: 24842846
. [PubMed] [Read by QxMD]
Sutton RM1, French B, Nishisaki A, Niles DE, Maltese MR, Boyle L, Stavland M, Eilevstjønn J, Arbogast KB, Berg RA, Nadkarni VM. American Heart Association cardiopulmonary resuscitation quality targets are associated with improved arterial blood pressure during pediatric cardiac arrest.
Resuscitation. 2013 Feb;84(2):168-72. PMID: 22960227
. [PubMed] [Read by QxMD]
Kleinman ME, de Caen AR, Chameides L, Atkins DL, Berg RA, Berg MD, Bhanji F, Biarent D, Bingham R, Coovadia AH, Hazinski MF, Hickey RW, Nadkarni VM, Reis AG, Rodriguez-Nunez A, Tibballs J, Zaritsky AL, Zideman D; Pediatric Basic and Advanced Life Support Chapter Collaborators. Part 10: Pediatric basic and advanced life support: 2010 International Consensus on Cardiopulmonary Resuscitation and Emergency Cardiovascular Care Science With Treatment Recommendations.
Circulation. 2010 Oct 19;122(16 Suppl 2):S466-515. PMID: 20956258
. [PubMed] [Read by QxMD]
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Evaluating the child with altered mental status can be very challenging. As with adults, there are a myriad of potential etiologies and it can be difficult to prioritize the order of the evaluation. Does this patient have an intracranial process or maybe just simple hypogylcemia? Or maybe we need to consider serious bacterial infections and intoxicants instead?
Many of you will recall the mnemonic “TIPS AEIOU” [Trauma, Infection, Psych, Space Occupying Lesion, Alcohol, Electrolytes, Insulin, Opiates, Uremia] to help consider a number of the potential etiologies of altered mental status. I use this often, but it is important to remember that along with “infection” and “insulin” the “I’s” also stand for “Intussusception.”
- Occurs when one segment of bowel telescopes into the adjacent segment.
- Second most common abdominal emergency (appendicitis is #1).
- Most often this occurs near the ileocecal valve.
- Most are idiopathic.
- ~5% are associated with lead points or disease
- “Classic” presentation (Abdominal Pain, Currant Jelly Stools, and vomiting) is only seen in 20%- 33% of cases.
- 75% without obviously bloody stools will have positive occult blood.
- Great reason to check heme occult for the vomiting child without diarrhea.
- Abdominal Mass is commonly found (when looked for).
- Fever, anorexia, diarrhea, and dehydration can be seen and can lead to misdiagnosis.
- Many cases are missed upon initial evaluation — we must always remain vigilant!
Intussusception and Altered Mental Status
- Along with the classic and common presentations, patients with intussusception may also present with altered mental status.
- This altered mental status is often thought of as the child being inconsolable.
- Most practitioners will have intussusception high on the DDx in the child with inconsolability and vomiting.
- Additionally, significant somnolence and lethargy can be seen with intussusception!
- Numerous cases have been reported that highlight this fact.
- Cause of the lethargy is unclear.
- Presence of lethargy does not, necessarily, portend a worse outcome (but does contribute to delayed diagnosis, which can lead to more morbidity/mortality).
- Cases of intussusception and altered mental status often also have history of:
- Heme-positive stool,
- Abdominal Pain,
- or Abdominal Mass
POCUS and Altered Mental Status
- So how can Point of Care Ultrasound (POCUS) help with the evaluation of altered mental status in a child?
- POCUS can be used to AUGMENT the physical exam.
- Studies have shown the POCUS can be used by emergency practitioners (in a variety of locales) to rule-in the diagnosis of intussusception.
- The evaluation should not (yet) be used to rule-out the diagnosis; however, it’s utility can help prioritize the next most appropriate step.
- For instance, consider the 18 month old presenting with lethargy and episodes of non-bilious emesis. You perform POCUS:
- If consistent with intussusception, then call your surgeon and your radiologist.
- If not consistent with intussusception, it may still be present, but you may want to get that Head CT and give antibiotics before getting abdominal imaging.
Moral of the Morsel
Altered Mental Status? Augment your exam with POCUS. It may be intussusception.
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Being flexible and creative are important traits to have while working the ED. It is also vital to always keep the end result in mind. Pain control is always an important endpoint for us to constantly consider. While most of us would say that we strive alleviate our patients’ pain, there is evidence that we are not great at it.
- Why would we not alleviate pain optimally? Well, like many things, it is more complicated than us just cruel and sadistic.
- Certainly, there used to be a perception that pain in kids (especially neonates) was not as important since they wouldn’t remember it. – WRONG.
- Additionally, there are times when our consultants have “requested” that we didn’t give pain medications (ex, Morphine for Appendicitis). – A MYTH.
- Of course, we need to always remain optimally educated on the subject (THANKS FOR READING THE PEDEM MORSELS!).
- But even with a highly educated and compassionate provider, delivering appropriate analgesics in a timely fashion is not easy:
- You have to evaluate the patient, then write the orders.
- Often these orders include Intravenous Analgesics (ex, IV Morphine).
- This, in turn, requires an IV.
- Now, in a busy ED, a nurse may not be able to promptly jump in that room an place the IV.
- Placing an IV is also not always an easy task.
- All of these steps and possible obstructions can easily lead to delayed analgesic administration.
- Now, despite how compassionate you are… you appear to be cruel!
Intranasal Route – No Need for an IV
- We all know that the blood supply to the nose is quite robust.
- Anyone who has bonked their nose knows.
- Our patients who snort heroine or cocaine also know.
- The venous drainage from the nose conveniently ends up in the SVC, avoiding the liver (and 1st pass metabolism).
- The anterior potion of the nose (the Vestibule) is the main site for drug absorption as it has a relatively large surface area and has a good blood supply.
- Volumes of 0.3 mL are easily tolerated.
- This requires concentrated solutions of the administered medications.
- If you need to use larger volumes, you can divide the dose in half and use each nostril.
- If the volume is still too large, you can administer in separate aliquots separated by 10-15 minutes… or use another strategy (nothing is perfect).
Intranasal Fentanyl to the Rescue
- Fentanyl is a great example of a medication that works well when given via the intranasal route.
- It has a low molecular weight.
- It is lipophilic.
- It has concentrated versions (50 microgr/mL – 150 microgr/mL).
- Fentanyl (1-2 micrograms/kg) given via intranasal route has proven to be as efficacious as IV Morphine (0.1 mg/kg).
- It has also been shown that intranasal fentanyl can be administered more rapidly than IV morphine to pediatric patients in the ED.
A Reasonable Approach (at least I think so)
- Intranasal Fentanyl can be delivered before even an IV can be placed.
- Even if you still need an IV (say for the grossly deformed forearm that you know will need procedural sedation), the intranasal fentanyl is still a faster way to get analgesics on board.
- Yes, it might require some explanation that you are going to squirt pain meds up the kid’s nose and then still place an IV… but the focus is on delivering pain meds quickly.
- This will likely also help the nursing team trying to get the IV, as now they have a more comfortable and cooperative patient.
- It also helps speed up the process for getting your X-rays… now you are not waiting for the IV to give the pain meds so that you don’t feel like a sadist getting the xrays.
- In the end, this also helps you… not feeling like a sadist is very helpful in avoiding compassion fatigue... and will help keep you happier as a physician!
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