Vacation can be so blissful! But, sometimes, that “EM mind” just won’t turn off. Have you, like a thirsty vampire, ever glanced at someone’s neck and admired the large external jugular veins? Do you pack your extra supply of suture material along with your bathing suit, just in case? When you drive by the vacation spot’s local hospital, do you wonder what their resources are? Well, if these things seem normal to you, then you, like me, are… slightly disturbed… and totally wed to being an emergency provider. Recently, I while floating on the lazy river, I saw a child who had obvious Marfan Syndrome features and my mind quickly sorted through some of the emergent conditions I should prepare myself to consider … again, … just in case. So let’s quickly review what my mind came up with for Marfan Syndrome.
Marfan Syndrome: Basics
- Marfan Syndrome is a disorder of the connective tissue.
- It is inherited in a AUTOSOMAL DOMINANT fashion.
- Frequency is at least 1 in 5,000 in the USA.
- ~ 1/4th of cases are due to spontaneous genetic mutation.
- There is no gender or ethnic preference or distinction.
- Effected protein = Fibrillin-1
- Important to the structure of connective tissue.
- Involved in connective tissue throughout the body.
- Normal fibrillin thought to inhibit growth of long bones and elastic fibers.
Marfan Syndrome: Clinical Features
- Variance in the expression of the condition exists.
- Not all patients will be affected the same.
- Features may be present at birth or develop later.
- May be diagnosed in adulthood.
- Patients diagnosed earlier appear to have better clinical courses than those diagnosed later in life. [Willis, 2009]
- Some of the clinical features are: [Kaemmerer, 2005; Pediatrics, 1996; Marfan.org]
- Pecuts excavatum or Pecuts carinatum
- Arm span:Height ratio >1.05
- Thumb sign
- Able to extend thumb beyond ulnar border of the hand when hand is flexed.
- Wrist sign
- Able to overlap the distal tips of the thumb and index finger when wrapped around contralateral wrist.
- Scoliosis >20 degrees
- Reduced extension of the elbows (<170 degrees)
- High arched palate with crowding of the teeth
- Aortic aneurysm
- ~50-83% of kids with Marfan syndrome have dilation of the aortic root. [van Karnebeek, 2001]
- Mitral valve prolapse
- Diagnosed at a mean age of 9.7 years. [van Karnebeek, 2001]
- Dilation of the main pulmonary artery
- Calcification of the mitral annulus in patients < 40 years of age
- Neonatal Marfans Syndrome presents with rapidly progressive and potentially fatal cardiovascular complications.
- Severe nearsightedness
- Early glaucoma / cataracts
- Flat cornea
- Apical blebs
- Asthma / reactive airway disease
- Lumbosacral dural ectasia
- May develop colonic diverticula at early age. [Santin, 2009]
Marfan Syndrome: The Emergencies
- Aortic dissection
- Obviously, this is the most feared and greatest concern!
- Fibrillin-1 is primarily expressed in the ascending aorta.
- Dissections typically in the second decade of life.
- There are other conditions that may require emergent evaluation and treatment:
- Occurs in ~5% of patients
- Cor Pulmonale
- May develop due to severe and progressive chest wall deformities and scoliosis leading to mechanical restrictions.
- Dislocation of the lens of the eye
- ~50-80% of cases have lens dislocation.
- Often the ophthalmologist may be the first to make the diagnosis.
- Retinal detachment
Moral of the Morsel
- Marfan syndrome affects many organ systems (not just aorta and bones).
- The patient with Marfan syndrome who is dyspneic may be sort of breath due to a variety of issues including reactive airway disease and mechanical issues, but don’t overlook pneumothorax!
- When your on vacation… close your eyes… otherwise you may start quizzing yourself on medical facts related to passersby’s pathology.
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Unquestionably, automobiles pose a significant health risk for both adults and children. Trauma related to car collisions are a leading cause of morbidity and mortality in children. Obviously this deserves much attention (Injury Prevention, Childhood Injury), but let us not forget that cars can present various other hazards for children. With the oppressive heat of summer dominating our days, let’s consider Vehicular Hyperthermia.
Hyperthermia: Contemplations for Kids
- Children are in the group of people who are at greatest risk for heat-related illness.
- Commonly cited reasons for this increase risk:
- Greater body-surface to mass ratio – adversely affects heat absorption
- Higher metabolic rate – kids generate more heat
- Lower perspiration rate – decreased heat dissipation
- Reduced acclimatization – adjust more slowly to environmental exposures
- Whether these traits truly influence a child’s susceptibility to heat exposure is debated. [Marshall, 2010; Rowland, 2008]
- What is known is that kids interact with their environment differently than adults.
- Older children, often don’t appreciate the danger their actions place them in (i.e., testosterone-laden teenage boys).
- Young children are dependent upon adults to keep them out of danger.
- Metabolic processes constantly generate heat.
- At rest, the body generates enough heat to raise the body temperature ~1 degree C/hr.
- Environment also influences the body’s temperature.
- When the ambient temperature exceeds the body’s, there is heat gain.
- Heat injury occurs when the body’s temperature rises faster than it can dissipate the heat.
- Despite numerous public service announcements (ex, kidsandcars.org, hyperthermia is still the leading cause of noncrash-related child mortality due to cars. [NHTSA.org]
- While the greatest risk is during summer months, it can occur year round. [Grundstein, 2015; Duzinski, 2014]
- The inside of the car can reach critical temperatures even during cold days. [Grundstein, 2015]
- In an infant model used to measure body temperature in a closed car, heat stroke temps were reached: [Grundstein, 2015]
- in hot months (28 C), in 105 min
- in mild months (17 C), in 200 min
- in cold months (1 C), in 315 min
- The best treatment is prevention!
- Always take simple opportunities to remind people of hazards that exist (ex, Detergent Pods, Lawn mowers)
- A sleeping infant can be easily forgotten by a overworked, exhausted, mentally distracted parent running a simple errand. Reminders of this can be powerful!
- ABC stabilization
- Cool the patient
- Spray the skin with room-temperature water.
- Direct electric fans onto the skin.
- Do not apply ice water widely to the body surface (may cause vasoconstriction)
- Ice packs to groin and axilla can be used.
- Invasive lavage is not currently recommended.
- Cooling blankets can be useful if available.
- Monitor core temperature
- Active cooling should be continued until temp is <39 degrees C.
- Hydrate with isotonic fluids
- Anticipate and treat complications
- Heatstroke affects all organ systems.
- Keep rhabdomyolysis on your Ddx.
- Transaminase levels correlate well with severity of injury and peak in 24-48 hrs.
- Monitor glucose levels closely. (Don’t let hypoglycemia fool you!!)
- Monitor coagulation studies to look for DIC.
- Some patients may benefit from venous-venous hemofiltration [Zhou, 2011]
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Summertime brings many delightful activities that had been left hibernating during the cold winter months. With each activity, however, comes a variety of injuries and illnesses. Certainly, we are aware of the potential injuries that come with activities like fireworks or skateboarding, but the summertime water activities bring their own unique issues like submersion injuries or C-spine injuries. Swimming can also lead to more minor, yet very annoying issues, like swimmer’s ear. Let’s take a moment to enjoy a morsel of Otitis Externa:
Otitis Externa: Basics
- Acute Otitis Externa (AOE) is a diffuse inflammation of the external ear canal. [Rosenfeld, 2014]
- AOE may involve the pinna or the tympanic membrane.
- AOE is actually a cellulitis of the ear canal skin.
- In North America, ~98% of acute otitis externa is due to bacterial infection.
- Most common bacterial causes:
- Pseudomonas aeruginosa
- Staphylococcus aureus
- Fungal infection is uncommon for acute otitis externa, but plays role in chronic otitis externa or in those who have been treated with antibiotics. [Rosenfeld, 2014]
- The cellulitis can spread and lead to complications.
- Cause of otitis externa is multifactorial: [Rosenfeld, 2014]
- Cerumen actually serves a purpose and efforts to remove it can increase risk of infection
- Skin disorders may create additional debris in canal that supports infection
- Local trauma to canal (often from cleaning attempts or hearing aids)
- Exposure to moist environment (ex, humid summer climate or swimming)
- Bacteria love to hang out in swimming pools and hot tubs!
Otitis Externa: Diagnosis
- AOE is uncommon in children <2 years of age. [Rosenfeld, 2014]
- Elements of the diagnosis include: [Rosenfeld, 2014]
- Rapid onset (often within 48 hours) AND
- Symptoms of ear canal inflammation:
- Otalgia (often severe) (seen in ~70%)
- Itching (seen in 60%)
- Fullness (seen in ~20%)
- May also have hearing loss or jaw pain (worse with jaw movement)
- Signs of ear canal inflammation:
- Tenderness of the tragus or pinna or both OR
- Diffuse ear canal edema or erythema or both
- May have otorrhea, regional lymphadenitis, TM erythema, or even cellulitis of pinna and adjacent skin.
- Tenderness of the tragus / pinna is often intense, even if visual inspection is underwhelming.
Otitis Externa: Ddx
- Acute otitis media w/ or w/o TM perforation
- AOM and AOE may both lead to erythema of the TM.
- Pneumatic otoscopy can differentiate – AOE will still have mobile TM.
- AOM with perforation will lead to debris in canal and mimic AOE.
- AOE will have very tender tragus and pinna while AOM w/ perforation often won’t.
- Malignant / Necrotizing otitis externa
- Agressive infection
- Predominantly affects patients with diabetes or other immunocompromised states
- 90% due to Pseudomonas aeruginosa
- Can lead to skull base osteomyelitis and further invade local structures (like the brain).
- Facial nerve paralysis may be early sign and is more commonly seen early in children vs adult. [Rubin, 1988]
- Look for granulation tissue on the floor of the canal and at the bony-cartilaginous junction. [Rosenfeld, 2014]
- Typically painless
- Has alterations of the TM (ex, retraction, granulation tissue, perforation)
- Need ENT referral for management
- Furunculosis (infected hair follicle on outer third of ear canal)
- Viral infections (ex, HSV – Ramsay Hunt syndrome)
- TMJ syndrome
- Skin disorders (ex, eczema, seborrhea, psoriasis) that involve the ear canal
- Contact allergy (ex, nickel allergy from jewelry)
Otitis Externa: Treatment
- Important to assess for factors that alter management strategies:
- Perforated TM
- PE tubes
- Immunocompromised states (ex, HIV)
- Prior radiation therapy
- Topical Antimicrobials are the main therapy!
- Initial therapy for uncomplicated AOE is topical antibiotics. [Rosenfeld, 2014]
- No clinical difference found between various options, although there is cost difference. [Rosenfeld, 2014]
- Typical course is for at least 7 days.
- If ear drops do not infuse easily, the patient may require a wick to be placed in the ear canal.
- If there is a suspected perforated TM or known PE tubes, avoid ototoxic agents!
- The middle ear does not have keratinized epithelium so drugs can pass through middle ear and into inner ear.
- Can lead to hearing loss.
- Need to avoid medicines with low pH, alcohol, aminoglycosides, or the combination drug neomycin/polymxinB/Hydrocortisone.
- In US, only quinolone drops are approved for middle ear use.
- Avoid systemic antibiotics [Rosenfeld, 2014]
- Oral antibiotics play no role in initial management of uncomplicated AOE.
- If there is extension of cellulitis outside of the canal or concerning host factors, then systemic antibiotics are needed.
- Malignant/necrotizing otitis externa requires systemic antibiotics and, possibly, anti-fungal medications in addition to surgical debridement.
- Do not forget analgesics!!
- The periosteum is very sensitive.
- NSAIDs to start with.
- Low dose opiates may be appropriate.
- Symptoms should improve within 48/72 hrs so prolonged courses of pain medications are not warranted.
- Topical anesthetic drops:
- May mask worsening disease, so great care should be taken if using them.
- Should not be used if PE tube or TM perforation is present/suspected!
- Reassess in 48 – 72 hours
- If no improvement in this timeframe, need to evaluate for other diagnoses.
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Infectious diseases seem to be omnipresent in the Ped ED and, thus, have their own dedicated Category of the PedEMMorsels. Additionally, unusual dermatologic eruptions are also quite prevalently encountered when caring for children (see Approach to Rash). While individually each category is fun to think about, the excitement more than doubles when they occur concurrently (perhaps I am being facetious). Let’s review Blistering Distal Dactylics! (say that 6 times fast in a row – I dare you)
Blistering Distal Dactylitis: What It Is
- Superficial infection of the distal finger (just like the name would imply)
- Tense bulla forms over the volar aspect / finger pad [Tessaro, 2016]
- May extend dorsally to the lateral nail folds.
- Oval shaped
- Erythematous base
- Often a singular lesion
- Filled with purulent material (yuk).
- Caused by:
- Group A Streptococcus pyogenes
- Most common pathogen
- Unclear how it causes bullae to develop
- Don’t forget other interesting Strep Infections:
- S. aureus
- Less common, but known to cause bullous disease (see Staph Scalded Skin)
- Multiple lesions may predict infection with S. aureus.
- Rarely is MRSA implicated. [Fretzayas, 2011]
- S. epidermidis
- Group B Streptococcus
- Typically affects kids 2 years to 16 years, but has been shown in children <2 years as well. [Lyon, 2004]
Blistering Distal Dactylitis: Ddx
- Herpetic Whitlow
- Bullous impetigo
- Insect bites
- Blistering disorders
- Dyshidrotic eczema
- Friction blisters and other mechanical irritants
Blistering Distal Dactylitis: Diagnosis It!
- Clinical diagnosis for the most part.
- Consider other etiologies like Herpetic Whitlow.
- May have concurrent infection at another remote site (ex, URI, pharyngitis).
- Multiple lesions suggests Staph as causative agent.
- Can confirm with testing:
- Gram-stain and culture of debris and fluid from blister.
- May perform rapid strep testing on fluid/debris. [Cohen, 2014; Wollner, 2014]
- Rapid Strep Test has similar test characteristics/performance as it does when applied to patients with pharyngitis.
Blistering Distal Dactylitis: Treat It!
- Local Wound Care
- No definitive recommendations for incision and drainage, but often the blister is unroofed to collect specimen. [Tessaro, 2016]
- Once unroofed, wet-to-dry dressings are appropriate.
- Systemic Antibiotics
- Coverage for strep and staph is paramount.
- Beta-lactamase-resistant antibiotic often selected.
- Empiric covered for MRSA is not likely beneficial at this point, but keep local resistance patterns in mind.
- 10 Day course often cited.
- Topical antibiotics alone are inadequate.
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The evaluation of pediatric patients with rashes is a common occurrence in the Ped ED… and a common area of frustration for many of us (ok, maybe it is just me). We have previously covered an approach to the evaluation of the Pediatric Rash. We have also covered a variety of common causes of pediatric rashes (ex, Scabies, Tinea, Diaper Dermatitis and Molluscum) including eczema. While many of these conditions are simple nuisances, some can become more problematic. Let’s make sure we stay vigilant for Eczema Herpeticum.
Eczema Herpeticum: Basics
- Atopic dermatitis is an inflammatory skin disease
- The inflammatory states creates an impaired skin barrier
- The impaired protection increases risk for bacterial and viral infections
- Eczema herpeticum
- A HSV skin infection that occurs in patients with atopic dermatitis.
- Occurs in 3-6% of patients with atopic dermatitis.
- Can be due to HSV1 or HSV2, but also other viruses can cause it (ex, varicella, poxvirus)
- May occur with either primary or recurrent HSV infection. [Wollenberg, 2003]
- More likely to occur in those patients with:
- Extensive eczema skin involvement
- Early onset of eczema [Wollenberg, 2003]
- Eczema lesions on head and neck
- High IgE levels
- Children who are young (1 year or younger) or have systemic illness (ex, fever) are more likely to require hospitalization. [Luca, 2012]
- Fortunately, overall mortality is low. [Aronson, 2013; Aronson, 2011]
Eczema Herpeticum: Presentation
- Systemic symptoms
- Skin eruption
- Monomorphic eruption of dome-shaped vesicles
- Initially starts in region of eczema
- Can spread to involve normal skin also
- Lesions may crust and form superficial pits and erosions
- Head, neck, and trunk frequently affected.
- Lesions can affect the eye and cause keratoconjunctivitis.
- Fluid from vesicles can be sent for HSV PCR or viral culture to confirm diagnosis.
- May have bacterial infection superinfection
- S. aureus is commonly cultured (~30%). [Aronson, 2011]
- Septicemia cases can occur, but less commonly (~3%). [Aronson, 2011]
- Dissemination of HSV
- Multiple organ involvement
Eczema Herpeticum: Treatment
- Acyclovir is the traditional therapy
- Depending on severity of condition, oral or IV is appropriate
- Oral acyclovir has low bioavailability, so only use for mild cases.
- Delayed administration of acyclovir in hospitalized patients is associated with increased length of stay. [Aronson, 2011]
- Each day of delayed initiation of acyclovir increased LOS.
- Challenging to recognize, but important to consider and initiate therapy early, similar to neonatal HSV.
- Topical steroids
- Concern that topical steroids may increase spread of HSV infection.
- Topical steroids are not definitively associated with worsening disease and prolonged LOS. [Aronson, 2013; Aronson, 2011]
- May be prudent to wait until acyclovir has been initiated. [Aronson, 2011]
- Systemic steroids do worsen eczema herpeticum and increase LOS. [Aronson, 2013; Aronson, 2011]
- Empiric antibiotics for all kids have not shown to improve outcomes. [Aronson, 2013]
- Early recognition of serious bacterial infection is important, however.
Moral of the Morsel
- Most rashes in children are benign, but remain vigilant.
- Eczema injures one of the body’s primary defenses against the outside world, so always consider bacterial as well as viral super-infections.
- If you see blisters in child with eczema, think Eczema Herpeticum!
- Obtain HSV PCR and viral culture of fluid from blisters and initiate acyclovir.
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