POTD- Sickle Cell

In todays POTD were gonna talk Sickle Cell Anemia. Why? Because we don't see it very often at maimo but complications related to SCA present pretty commonly to most EDs... and because I randomly had about 10 Rosh review questions in row about it...

We all know the basics at least vaguely from step 1

• 100,000 ppl in US, 2mi Carriers. Mc African, Mediterranean, Indian and Middle Eastern
• AR exchange of Valine for glutamic acid in the B global chain changing the structure so that when deoxygenated it sickles
• Diagnosed on newborn screen or after 4mos of life (Why? because of the decline in fetal Hb)

So lets talk some of the more common presentations:

• Remember basic workup should include CBC and retic count plus specific labs/imaging for sx..

Stroke:

• RFs: High flow velocity on dopler, low Hb, high WBC, HTN, hx TIA, hx ACS
• Imaging: PEDS: NEED MRI w/ diffusion as CT can miss and early infarct!
• Trx:
  • Adult: tPA v intraaterial thrombolysis
  • Peds: IVF and exchange transfusion with goal of HgbS < 30%
    • Target MAX HG of 13 or can cause recurrent ischemia
    • 30% have hemorrhagic transformation
    • *** Control BP with labetolol to goal of 50-95th% for age

Acute Chest Syndrome:

• Fever, cough cp, hemptysis , dyspnea
• CXR: new infiltrate
• Some Guidelines:
  • All pts should be hospitalized for pain control and monitoring
  • Trx with IV cephalosporin + oral macrolide
  • Goal O2 >95%
  • Transfuse if Hb is >1g/dL below baseline
  • Higher mortality in adults so should go to ICU as can progress to ARDS

Pain: The somewhat dreaded hard to control complain,

1st and foremost be sympathetic and treat accordingly, they often have their own pain plans and can provide some insight into what works.

• Think about these dangerous causes and don't just dismiss as "drug seeker"
  • CP: Think ACS v ARDS Vhighoutpt heart failure v PNA v pain crisis
  • HA: Think stroke v meningitis v CVST v ocular pathology
  • Arthralgias: Septic arthritis v actor trauma v avascular necrosis
  • Abd Pain: Splenic sequestration v acute intrahepatic sequestration v pain crisis 2/2 occlusion of mesentery v renal infarct
• Treating Pain:
  • Should get analgesia w/in 30min of arrival
  • Use NSAIDs as adjunct
  • Follow pts pain plan if available
  • Avoide Meperidine

Fever:

• MCC? Viral, But always think of the encapsulated organisms (S pneumonia, HiB, non-typhi Salmonella, Mycoplamsa, C. Pneumonia, Yersinia) which are higher risk in these pts d/t functional asplenia
• Prophylactic PCN for pts 2mo-5years- check compliance, PCV at 2mos, Influenza vaccine at 6mos
  • Bactermia, Pneumonia--> S pneumonia
  • UTI--> EColi
  • Osteo--> Salmonella
  • TRX: Ceftriaxone +Vanco
• Some great guidelines from CHOP for pediatric fever: http://www.chop.edu/clinical-pathway/sickle-cell-disease-with-fever-clinical-pathway

A couple others...

RUQ pain: Acute chole v cholelithiasis v acute intrahepatic cholestasis v acute sickle hepatic crisis v acute hepatic sequestration

• CBC, LFTs, Coags, imaging- CT v US
• Trx of AHS is simple v exchange transfusion

Splenic Sequestration:

• Acute drop in hgb >2, splenomegaly, reticulosytosis, intrascaular volume depletion
• Rapid progress to shock and death
• MC kids 10-27 mos old
• IVF + transfusion ( simple v exchange)

and don't forget the beloved board question... Aplastic Crisis... from what Parvovirus B19

• Pallor, tachycardia, supportive care unless retic < 1-2 then simple transfusion.

A great summary chart!

AND FINALLY... When to transfuse:

Sources:

JAMA 2014 Sep- Manamge of SCD: summary of the 2014 evidence-based report by expert panel members.  https://www.ncbi.nlm.nih.gov/pubmed/25203083

EMDocs

CHOP clinical guidelines

RoshReview

Trauma Tuesday: Burr Holes!

Hi everyone, This Trauma Tuesday, we'll be discussing the ED approach to Burr holes, inspired by a discussion from today's Trauma Sim. This is admittedly a procedure that few if any of us have ever or will ever need to do. But just in case you ever find yourself manning a rural ED with the nearest neurosurgery center hours away, let's refresh our minds.

There was a great article in last month's ACEP Now newsletter (http://www.acepnow.com/article/perform-emergency-burr-hole-procedure/) written by the medical director of a rural 12-bed ED who recently saved a young boy's life by performing a Burr hole:

2 year old child presented after falling out of shopping cart. Initially appeared well and running around the exam room. However, he became somnolent after a period of observation and had slightly unequal pupils on repeat exam. Head CT showed a large epidural hematoma with midline shift. His pupillary exam drastically worsened (6 mm and 2 mm) and he was intubated. The nearest pediatric trauma center was 1 hour away by helicopter. The patient would almost surely herniate en route if no intervention was done. The physician made the decision to perform a Burr hole, evacuated 150 mL of blood, pupils improved, pt was shipped out, and returned to the ED 1 month later for an unrelated visit, running around and completely neuro intact. PRETTY AWESOME, RIGHT?

First let's review the indications for an ED burr hole. They're pretty simple:

• Epidural/subdural hematoma with midline shift on imaging and unequal pupils on exam
• GCS<8
• Anticipated extended time to neurosurgical intervention. Small studies show that ED Burr holes are most effective when performed within 60-90 min of onset of anisocoria. (Door-to-drill time metric?? Think of those patients who arrive walky/talky and decompensate in front of your eyes. Sort of reminiscent of indications for an ED thoracotomy)

Next, let's review the anatomy. This diagram shows 3 potential locations for Burr holes to be done, depending on the location of the bleed on CT. I've seen Neurosurgery in our ED use the parietal site to place an external ventricular drain. The safest location for the ED physician is to go for the temporal site, due to lowest risk of further puncturing the middle meningeal artery. The temporal site is found by going 2 cm anterior and then 2 cm superior to the tragus (pictured).

Now let's review the procedure itself.

STEP 1: Get your equipment - manual trephinator (commercially made kits are available; the Galt trephine shown below is typical), sterile drapes/gown/gloves, chlorhexidine preps, razor (must shave the scalp at the site), scalpel, local anesthetic.

STEP 2: Adjust the stopper on the trephinator to the appropriate depth based on the CT, as shown here:

STEP 3: Once the site is shaved and prepped, inject local anesthetic. Start with a vertical incision with the scalpel down to the periosteum to expose it.

STEP 4: Apply the trephine with gentle, steady pressure until the skull is penetrated. Remove the bone fragment and store in sterile saline. The clot may not immediately extrude; if so then use a pediatric suction catheter to GENTLY facilitate hematoma drainage. Once blood flow slows/stops, apply a loose sterile dressing. Do not tamponade the bleeding.

And of course, final step is to transfer the patient out immediately. Who's ready to do some Burr holes? :)

References: https://wikem.org/wiki/Burr_hole http://resus.me/burr-holes-by-emergency-physicians/

Ankle Dislocations

Happy New Years Eve everyone! Before people head out for some NYE festivities lets talk Ankle Dislocations!

One was seen in the ED this week and managed beautifully from what I hear, so lets make sure we all know what to do as this is an orthopedic emergency!

Mechanism:

• Mc= Post dislocation of talus can be medial, ant, lateral and superior
• HIGH force injury --> plantar flexion
  • Greatest instability as talus becomes narrower
  • Inversion? posteromedial discplacement + injury to ATF and CF Ligaments
  • Eversion? Lateral dislocation
• Fall with axial load, car accident
• mc Young males, pt with previous ankle injuries, Ehrles Danlos

 

Complications:

• High association with fractures
• High risk of Neurovascular injury so need FAST RECOGNITION AND REDUCTION!
  • Can lead to avascular necrosis of the talus, sensation loss and LE tissue necrosis--> gangrene
• Nonunion/malunion, tendon entrapment, cartilaginous injury chronic arthritis, rarely a/w compartment syndrome

Work up:

• PE: Edema, tenting os skin, tip along joint line, deformity
• ALWAYS CHECK FOR PULSES and SENSATION
• XR- A/P, Late and Mortise views

When to Reduce:

• Indications:
  • NV compromise- Just reduce! don't need X-rays 1st if high clinical suspicion
  • No compromise- confirm with X-ray first
    • Can be open, can be a/w fracture

• Contraindications:
  • Multiple failed attempts
  • Subtalar Dislocation
    • Rare, high force on forefoot
    • 20% are irreducible and need OR

Reduction

• Pre-Procedure:
  • Sedation and pain control is key
  • Have material ready to cast following reduction
• POST:
  • Hold foot in plantar flexion, apply axial traction
  • Posterior traction to to ditasl tibi and ant pressure to heel
  • https://www.youtube.com/watch?v=ANA2b-g3qaw
• ANT: Same 1st steps but apply anterior traction to distal tibia and posterior force to foot

Post Reduction:

• Immbolize with LONG LEG POST splint w/ SUGAR TONG component
• Repeat X-ray
• Can cause conversion to open injury during reduction--> give Tdap and abx
• Ortho Follow up for ORIF

Surgical Indications:

• Failure to reduce x 2-3 attempts
• Increasing tension or tenting of skin
• multiple other intra-articular fractures, subtler dislocation
• Amputation

Sources: OrthoBullets, Medscape

Wishing everyone a happy and healthy New Years, see you in 2018!

Julie