Case of the Week COW #12

CC: Left leg pain

HPI: 52 year old male with PMH of IDDM presents to the Emergency Dept. (ED) with left leg pain for the past 9 days which has become progressively worse. He is a taxi driver and reports that a part of the seat, which supports his legs, has been rubbing against his left hip/buttock/thigh and he thinks this is what’s causing the pain. He reports pain to the back of his left buttock, which radiates down the leg “like a shooting pain.” The patient has been to the ED multiple times already for similar complaints, requesting for stronger pain medications. He is upset because he feels the medicine just isn’t working. At this point, the patient is uncooperative and refuses to answer any more questions. Further history was obtained from his previous visit history, which stated he was discharged yesterday with a diagnosis of sciatica and a prescription for Lidoderm patch, Motrin and Percocet.

PMH/Birth History: N/A

Social History: N/A, refused to answer any further questions

PHYSICAL EXAM

VS: BP 125/59     HR 108     RR18   T: 98F   98% RA

General: In moderate acute distress, appears stated age, in moderate pain, uncomfortable and diaphoretic.

HEENT: Atraumatic, normo-cephalic. No deformities. PEERLA

Respiratory: Lungs CTA bilaterally.

Cardiac: +S1/S2, no MRG, regular rate and rhythm

Abdomen: soft NT ND

Extremities: B/L DP 2+, Cap refill < 3 seconds, positive straight leg (LLE); pain isolated to (L) buttock and posterior lateral thigh.

Neuro exam: CN III –XII intact. 5/5 strength in all 4 extremities with limited ROM in the LLE secondary to pain.

Skin: Diffuse erythema over the Left buttock extending down to the posterior lateral left thigh, with pitting edema. No fluctuance or streaking noted.

Labs:

WBC: 24.7     H/H: 13.1 / 39.4     Platelets 245     PMH: 22.1  Lymph: 0.7     Mono: 1.5

Na: 126     K: 5.5                 Cl: 89       CO2: 22           Glucose: 438

Bun: 38     Cr: 1.17         Alk P: 140       LFT: WNL         Albumin 3.2

Acetone: NEG

ESR: 65

CRP: 30

Lactic acid: 1.4

Images:

Repeat Vital Signs 139/81   89   16     99.7F   97% ON RA

MRI

 

 

Working Differential Diagnosis: Pyomyositis

ED/Hospital course:  Orthopedic Surgery was consulted. X-Ray did not show obvious bony involvement and ESR/CRP was not suggestive of osteomyelitis. MRI was performed on the LLE, which sowed a hyper-intense signal within multiple muscles of the pelvis and left thigh consistent with myositis. Collection within the Obturator Externus and Gluteus Maximus muscles likely represented an abscess consistent with pyomyositis. The patient was started on Vancomycin and Zosyn and sent to Interventional Radiology for drainage of the abscess. The cultures grew back MSSA. The left knee tap did not grow any organisms on Gram Stain. IR drainage was followed by orthopedic washout and debridement of the musculature with insertion of JP drain for continuous drainage. The patient’s antibiotics were switched to Levaquin and he was subsequently discharged home with Clindamycin and Bactrim for 2 more weeks. He tested negative for HIV.

Pearls & Takeaways:

  1. Don’t blow off patient’s complaints! Our patient presented with History and Physical exam consistent with sciatica with a positive straight leg test. He was on Percocet and kept asking for stronger medications for his previously diagnosed Sciatica.
  2. Make sure to undress the patient and examine the skin!
  3. If the patient is complaining of pain out of proportion to his/her exam, dig a little deeper for alternative differential!
  4. Since his Accucheck was High, further laboratory testing was done which revealed leukocytosis. The elevated white count prompted me to perform a further work up
  5. Always re-evaluate the patient! On re-evaluation, he appeared sicker and with cool, damp skin on his back and neck, solidifying my gestalt that maybe I am missing something
  6. Pyomyositis is a purulent infection of skeletal muscle that arises from hematogenous spread, usually with abscess formation.
  7. Risk Factors: immunodeficiency (HIV).
  8. auerus is the most common cause of pyomyositis; it causes up to 75 – 90% of cases.
  9. Pyomyositis presents with fever and pain and cramping localized to a single muscle group. It develops most often in the lower extremity (sites include the thigh, calf and gluteal muscles) but any group of muscles can be involved including iliopsoas, pelvic, trunk, Paraspinal and upper extremities.
  10. MRI is the most useful imaging modality for diagnosing the disease. It can distinguish the defining sites of infection and rule out other entities.
  11. Nonspecific lab findings include Leukocytosis and elevated inflammatory markers but CPK are often normal.
  12. Empiric antibiotics should be directed against Staph/Strep for immunocompetent. Immunocompromised should be covered for Gram negative, gram positive and anaerobic organisms should be considered.
  13. Pyomyositis is graded based on stages.
    • Stage 1 (Invasive stage, 1-2 weeks, may only have pain) can be treated with Antibiotics alone.
    • Most patients present with Stage 2 (Suppurative stage, weeks 3-4)
    • Stage 3 (Late stage) due to delay in diagnosis and usually requires drainage for definitive management.

Case presented by Dr. Michael Hong

Pain Pearls – Nitrous Oxide

Nitrous Oxide


Introduction to Nitrous in the ED

  • Tasteless colorless gas administered in combination with oxygen
    • Maximum concentration 70% N20
  • Absorbed via pulmonary vasculature and does not combine with hemoglobin or other body tissues
  • Rapid onset and elimination
    • <60 seconds
  • Analgesic and anxiolytic agent
    • Use along with local anesthetic or other non-opioid pain medications
  • Administered via facemask
  • Only monitoring needed is pulse oximetry
  • No NPO requirements, patient can drive after administration, no IV line needed, no RN necessary at bedside
  • Nitrous is NOT procedural sedation and should be thought of as a pain medication
  • Studies show it is similar to morphine 10 mg IM
  • It works only when the patient is breathing it in and stops working immediately when the gas is off

Contraindications

  • Severe COPD or asthma
  • Active Otitis Media/Sinusitis
  • Active Bowel Obstruction
  • 1st -2nd trimester pregnancy
  • Altered level of consciousness
  • Severe Psychiatric disease
  • EtOH
  • Head Injury
  • Complex Advanced Active Cardiac Disease

Indications (really any procedural pain)

  • Laceration repair
  • Incision & Drainage
  • Wound Care
  • Foreign body removal
  • Central Venous Access
  • Peripheral venous access
  • Fecal Disimpaction
  • Adjunct for
    • Dislocations
    • Splinting

Nitrous as an adjunct

  • If using nitrous to try and reduce a joint
    • Use IN ketamine
    • Intra-articular lidocaine
  • Concomitant admin of nitrous with opioids, benzos, or propofol is considered PSA
  • If giving an IV opioid you should wait 30-45 minutes before using nitrous to avoid excessive sedation or airway issues

What do we have?

Porter Standard Flowmeter

  • Stored in the trauma bay
    • Extra tank rack across from peds resus bed
  • Must use disposable breathing circuit
    • Located next to desk in trauma bay
    • There are adults small, youth medium, and peds small
Safety Features
  • Nitrous oxide flow stops if oxygen is shut off or supply becomes limited
    • Can never supply more than 70% nitrous oxide
  • Exhaled gas is scavenged away to avoid unnecessary exposure to providers

Getting started

  • Wheel to bedside
  • Plug green oxygen tube into oxygen port in the wall (may have to remove Christmas tree)
  • The gray tube scavenging tube plug into to the suction port in the wall
  • Breathing circuit only fits one way, push big lumen tube into top (by gray bag) and the clear little tube below breathing bag

Positioning

  • Position the patient comfortably
  • Bring unit close to the patient so you can reach the dials
  • Make sure pulse oximetry is connected and you can see the waveform
  • Once the mask is on…
  • Set oxygen flow rate then titrate nitrous oxide to desired percentage
  • Watch gray breathing bag as a guide to adjust flow (titrate flow)
    • If bag is fully collapsing, increase overall flow
    • If bag is not moving, decrease overall flow
    • Once nitrous oxide percentage is set, regardless of flow rate, percentage stays the same

During The Procedure

  • Titrate nitrous oxide up and down as needed for “desired analgesia”, watch breathing bag to determine overall flow
  • Once procedure is complete, turn off nitrous oxide, allow patient to breath oxygen only for 60 seconds
    • Continue to watch breathing bag
  • Then remove mask completely

Your Done!!

  • Patient can be discharged with no driving or work restrictions
  • Throw away the breathing circuit
  • Return it to the trauma bay

REFERENCES:
  • Becker 2008
  • Babl 2015
  • Zhang 1999
  • Champman 1979
  • Jastek 1991

Special thanks to Dr. Alexis LaPietra for her contribution! Watch out for next times pain pearls…….. Haldol as an analgesic!!!

Case of the week COW #10

CC: Nausea and vomiting and “ I think my sugar is low”

HPI: 36-year-old Female with PMH of Diabetes presents to the Emergency Department complaining of multiple episodes of non-bloody, non-bilious vomiting for the past 5 days. Associated symptoms include chills and a mild sore throat. Denies fever, HA, neck pain, chest pain, SOB, abdominal pain, diarrhea or bloody stools. Denies dysuria, hematuria or urinary frequency or urgency. Denies recent travels or sick contacts.   LMP: Currently menstruating

PMH: Diabetes

PSH: C-Section

Meds: None

Allergies: NKDA

Social: Admits to smoking 2 cigarettes per day for the past 10 years. Smokes marijuana daily. Denies alcohol use

 

Physical Exam : 

BP 171/82   HR 82    RR 20     SpO2 100% on RA    Temp 97.8F     108.86 kg

Constitutional: Alert, wake, and in no acute distress. Obese

HEENT: NCAT, pupils PERRLA, neck is supple. Oral mucosa is pink, dry and intact. No lesions. Normal conjuctiva

Respiratory: CTA B/L, no rales, rhonchi, no stridor or wheezing

Cardiac: +S1/S2, regular rate and rhythm, no murmur, rubs or gallop

Abdomen: Soft, with mild tenderness in epigastric area. No rebound or guarding. Normal BS

Neuro: AAO x 3. No focal deficits, moving all four extremities. Steady gate without difficulty.

MSK/Extremities: no edema, tenderness or swelling.

Skin: Moderate ecchymosis overlying RUQ and RUE. Normal moisture. No rash or lesions noted

 

Pertinent Labs:

WBC: 8.4   HGB: 9.3   HCT: 26.8   Platelet: 9L

Na: 136        K: 3.4          Cl: 104        Co2: 26      BUN: 16     Cr: 0.95   Glucose: 109

PT: 14.4   PTT: 28.7     INR: 1.1

Calcium 8.8         Total Bilirubin: 2.8       Alk Phos: 56           AST: 21                 ALT: 12

Lipase: 60

UCG: negative

Urinalysis: Large Blood with negative Nitrites and Leukocyte Esterase without ketones. UA Protein: 100

 Pertinent Images and other tests: Chest X ray: Borderline heart size. NO active disease

Upper Abdominal U/S: Normal Liver and spleen. The spleen measured 10.9 cm in greatest dimension and was homogeneous in echotexture. No focal splenic lesion identified. The liver measured 16.5 cm and appeared normal.

EKG:

 

Working Diagnosis:

Thrombotic Thrombocytopenia Purpura (TTP)

Idiopathic Thrombocytopenic Purpura (ITP)

Hemolytic Uremic Syndrome (HUS)

Anemia due to blood loss

Infection/Sepsis

ED Course: On further questioning, patient states she has been menstruating for 3 weeks now and bleeding is heavier as compared to the previous cycle. In the ED, patient was given NS 0.9% 1000mL IV bolus once, Zofran 4 mg IV once, Reglan 10mg IV once. Type and screen and blood cultures were also sent and patient was admitted to the Medical ICU for thrombocytopenia and anemia.

Hospital Course:

In the Medical ICU, further lab results demonstrated elevated the following:

LDH: 699         Fibrinogen: 410     Haptoglobin: < 10              Troponin 0.44

Blood Cultures: No Growth

Urine Toxicology: Positive for cannabinoids

Hepatitis C antibody: < 0.1

HIV Ag/Ab: Non reactive

In the ICU, patient was given a 125mg bolus of IV Solu-Medrol and then started on 60mg IV every 8 hours. She was transfused 1 Unit of platelet with no response, as the following day, platelets actually dropped to 5.Hematology/Oncology was also consulted. They stated that the occasional schistocytes seen on blood smear with low Haptoglobin levels and elevated reticulocyte count and LDH was suggestive of microangiopathic hemolytic anemia, making TTP a more likely diagnosis. Heme/Onc recommended against any further platelet transfusion and ADAMS 13 levels were sent. On hospital day #3, patient’s LDH levels had increased to 1586 and Troponin was elevated to 1.2 with symptoms of dyspnea. Patient also had worsening anemia as hemoglobin dropped to 7.0, and platelets remained at 9. The same day, Surgery was consulted and patient was transfused 2 Units of PRBs and 2 Units of FFP in preparation for Shiley Catheter insertion for plasmapheresis. Cardiology was also consulted for the elevated troponin, which they attributed to ongoing demand ischemia without any evidence of ACS. Unfortunately, the following night at 1:25 AM and before a Shiley was ever placed, the patient became minimally responsive and Saturating at 90% on RA. Immediately, she became bradycardic and lost pulses. CPR was initiated as patient was simultaneously intubated. Total duration of CPR was 60 minutes. The patient received Epinephrine x 20, Amiodarone 300 mg x 1 and Insulin with 1 amp of D50W, Calcium chloride and Sodium Bicarbonate for Potassium of 6.1 on ABG. Sadly, the patient was pronounced at 2:25AM.

PEARLS : Thrombotic Thrombocytopenic Purpura (TTP)

PENTAD = ‘FAT RN”

  1. Fever (50%)
    • Uncommon if they present early in the disease
    • High fever and chills suggest sepsis, so look for a source of infection.
  2. Thrombocytopenia
    • MAJOR diagnostic criterion
    • Mean platelet of 25, 000 but they reach 5000.
  3. Microangiopathic Hemolytic Anemia
    • MAJOR diagnostic criterion
    • Non-immune hemolysis with schistocytes
    • Results in elevated LDH, Low haptoglobin and high indirect bilirubin
    • Severely elevated LDH has been associated with MI, arrhythmias, shock and heart failure.
  4. Renal Failure
    • Due to renal thrombotic microangiopathy
    • Urinalysis will show mild proteinuria +/- hematuria
    • Acute Rena insufficiency may be present and may require dialysis.
  5. Neurological Symptoms: (Seizure, AMS, HA, coma, hemiplegia, aphasia, etc.)
    • Focal Deficits less prevalent
    • Occurs in 25-60% of patients
    • Symptoms are usually transient and subtle, so you must ask the patient about prior symptoms!

*** KEEP in mind; all features of the PENTAD do NOT to be present at the same time for diagnosis to be made.

Risk Factors

  • Obesity
  • African American race
  • Female
  • Ages 30-50 years
  • HIV/AIDS
  • Rheumatologic/autoimmune disease history

 Clinical Presentation

  • Fatigue, tachycardia, pallor, SOB or chest pain
  • Abdominal pain, back pain, Nausea, vomiting or diarrhea
  • New Onset jaundice and dark colored urine

Physical Exam

  • Diffuse, non-palpable petechial/purpuric rash.

Etiology

  • ADAMTS-13 protease enzyme deficiency
    • 60% of cases
    • Enzyme cleaves von Willebrand factor (vWF) multimers. Without it, excessive platelet aggregation, thrombocytopenia and thrombosis occurs
  • Idiopathic or Secondary Causes
    • 40% of cases
    • (i.e. HIV, malignancy, Infection, Pregnancy, pancreatitis, autoimmune disease, pancreatitis and medications such s Acyclovir, Quinine, Oxymorphoine, Plavix and Tacrolimus)
  • Diagnosis/Lab findings
    • NORMAL PT, PTT and Fibrinogen levels (Unlike in DIC)
    • Thrombocytopenia
    • Anemia and elevated indirect bilirubin
    • A peripheral smear is important for diagnosis, as 100% of patients will have schistocytes present during the course of the disease
    • ADAMTS-13 activity < 10% (normal activity is > 50%)

*** Remember that TTP is a clinical diagnosis. Do NOT delay treatment!

  • Differential Diagnosis
    • Other Causes of Microangiopathic Hemolytic Anemia (MAHA)
      • DIC, HUS, HELLP Syndrome, Malignant Hypertension, Heparin induced thrombocytopenia, Paroxysmal Nocturnal Hemoglobinuria, etc.
    • ITP
    • Sepsis
    • SLE
    • Viral infections (HIV, mumps, varicella, EBVS)
  • Management
    • Consult Hematology!
    • AVOID Platelet Transfusion if possible!
      • It can lead to renal failure, higher rates of arterial thrombosis and death
      • Should be AVOIDED EXCEPT in life-threatening bleeding or ICH
    • Plasma Exchange (Plasmapheresis) is First Line treatment!
      • Replaces defective or insufficient ADAMTS-13 and clears vWF multimers
      • LDH can be used to evaluate for treatment response
    • FFP Transfusion
      • Contains ADAMTS-13
      • Can be helpful if delay in plasmapheresis
    • Transfusion of RBCs (indicated ONLY in severe bleeding with a delay in plasma exchange)
    • Glucocorticoids
      • Adjunct Treatment and do not replace plasmapheresis
      • 1mg/kg Prednisone PO or Solu-Medrol 125mg IV
    • IVIG
      • Not first line but may be used in those who fail plasmapheresis
    •  Splenectomy
      • Last line therapy after stabilization
      • Inhibitor antibody is made in the spleen

Suspect TTP in any patient with MAHA and thrombocytopenia!

Case presented by Dr. Yenisleidy Paez Perez, DO

EM Conference Pearls (9/20)

Pediatric congenital heart disease

  • Congenital HD: Two types: Neonates with ductal dependent lesions and infants (2-6months) presents with CHF
  • Cyanosis presentation: When ductal-dependent lesion is required for pulmonary blood flow (Will not respond to oxygen)
  • Shock presentation: When ductal-dependent lesion is required for systemic blood flow (appear septic and not response to fluids, may get worse with fluids)
  • Hypoxic/cyanotic or shocky/acidotic baby treatment = Prostaglandin E1 (PGE1) and transfer to facility with pediatric cardiovascular surgeon.
  • PGE1 treatment may cause apnea (monitor closely and consider intubation)
  • CHF in infants = wheezing, retractions, tachypnea, sweating/crying, difficulty feeding

EBM in the ED

  • EBM = What the evidence shows in the literature + What the physician wants for the patient + what the patient wants for themselves
  • Just like we need to practice intubation, central lines –> Learning to read and interpret literature is a skill that needs to be practiced.

Aortic Dissection

  • AD: Chest pain plus disease (ex: CP + Neurodeficit)
  • If you find your self giving large amounts of strong pain meds (narcotics) while treating what is seemingly ACS…STOP..think about AD or alternative diagnosis of chest pain
  • The 3 important questions, aortic dissection is the subarachnoid hemorrhage of the torso, migrating pain, colicky pain + opioids = badness and pain that comes and goes can still be a dissection.
  • Treatment: Treat pain, HR, BP
  • Pain: Fentanyl 25 – 50 mcg bolus
  • HR: Goal of 60 bbpm
  • Esmolol 0.5 mg/kg bolus then 50 – 300 mcg/kg/min or
  • Labetalol 10 – 20 mg bolus then 0.5-2 mg/minor
  • BP control: Goal SBP =110
  • Nitroprusside 0.25 – 0.5 mcg/kg/min then titrate (CN toxicity)
  • Nicardipine 5 mg/hr
  • Warning: Giving a vasodilator without concomitant reduction in ionotropy may cause progression of dissection. Start BB first before vasodilation meds.

Case of the Week COW #7

CC: Numbness and palpitations

HPI: 21-year-old Female presents to the Emergency Department (ED) complaining of palpitations and left arm weakness with perioral numbness, which began just prior to arrival. The patient states the episode lasted 30 minutes before completely resolving on its own. In the ED, she denies any other complaints except for a mild headache. Patient notes she experienced a similar episode of palpitations yesterday afternoon, while resting, which she described as “skipping beats”. On further questioning, the patient admitted to being hospitalized to a NYC hospital 2 weeks ago where she had a Cardiac Echocardiogram done which showed “hypertrophy.” Patient never followed up with cardiologist as instructed. In the past, a doctor in her country prescribed her an unknown antihypertensive medication, which she took for one year but stopped taking it once she moved to NJ. Denies fever, dizziness, chest pain, and shortness of breath, recent travels, calf pain or swelling.

Physical Exam:

BP 109/72   HR 82     RR 18     SpO2 100% on RA    Temp 97.0F

General: Well appearing female, in non-acute distress

HEENT: NCAT, pupils PERRLA, neck supple

Respiratory: CTA B/L, no wheezing, rales or rhonchi

Cardiac: +S1/S2, no MRG, regular rhythm

Abdomen: soft NT ND

Neuro exam: AAO X 3, No focal deficits

Extremities: no edema, no tenderness or swelling, 5/5 strength in all extremities. Sensory intact

Skin: pink and warm, No diaphoresis, no rashes, lacerations, or abrasions

Pertinent Labs: Troponin 0.308

Pertinent Imaging and other tests:

  • Chest X-Ray: Cardiomegaly with a boot shaped heart, which may indicated right heart failure.
  • CT Head: Normal 
  •  ECG: Normal Sinus Rhythm, Bi-atrial enlargement, RBBB, LVH

Working Diagnosis: Hypertrophic Cardiomyopathy (HCOM)

ED/Hospital course:  Patient was given 324 mg of ASA and admitted to Telemetry with a diagnosis of Hypertrophic Cardiomyopathy. While still in the ED waiting for a bed on Telemetry the patient had multiple runs of non-sustained V-Tach and Cardiology was consulted. The patient was started on ASA and Metoprolol PO. A 2D ECHO was done which was consistent with HCOM. Patient remained stable on Telemetry for 3 days prior to discharge. The patient’s Troponin was trended daily, 0.308 in the ED, 0.288 on day 1 of admission, and 0.314 on day 2 of admission. Patient was told to follow up with Cardiology Clinic for possible AICD placement planning.

Pearls:

Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiac disorders (affecting ~ 1 in 500 people) and is the number one cause of sudden cardiac death in young athletes. Annual mortality is estimated at 1-2 %.

  • Pathology and Pathophysiology:
    • Dynamic Obstruction of the Left Ventricular Outflow Obstruction (LVOT)
    • Primarily Autosomal Dominant Inheritance
    • Left Ventricular diastolic dysfunction resulting from impaired relaxation and filling of the stiff and hypertrophied left ventricle (often associated with increased filling pressure)
    • Abnormal intramural coronary arteries with thickened walls and narrow lumens
    • Chaotic, disorganized left ventricular architecture (“ cellular disarray’) predisposing to abnormal transmission of electrical impulses and thus serving as a substrate for the formation of arrhythmia.
  • Clinical Manifestation
    • Exertional syncope or pre-syncope – this is the most worrisome symptom, suggesting dynamic LVOT obstruction with or without dysrhythmia, with the potential for sudden cardiac death.
    • Symptoms of pulmonary congestions due to left ventricular dysfunction (e.g. exertional dyspnea, fatigue, orthopnea, paroxysmal nocturnal dyspnea)
    • Chest pain – may be typical angina pain due to increased demand (thicker myocardial walls) and reduced supply (aberrant coronary arteries).
    • Palpitations due to supraventricular or ventricular arrhythmias.
  • ECG Features:
    • Left atrial enlargement
    • Left ventricular hypertrophy with associated ST segment / T-wave abnormalities
    • Deep, narrow (“dagger-like”) Q waves in the Lateral (V5-6, I, aVL) and inferior (II, III, aVF) leads. Most common in Lateral vs. Inferior
    • Giant precordial T-wave inversions in apical HCM
    • Signs of WPW (Short PR, delta wave)
    • Dysrhythmias: Atrial Fibrillation, supraventricular tachycardia, PACs, PVCs, VT

*** Infarction Q Waves are wider with a different morphology compared to HCM.

Pathologic Q Waves:

  • Usually > 40 ms (1mm) wide
  • > 2mm Deep
  • > 25% of depth of QRS Complex
  • Best seen in V1-V3

 

Post by Tyler Manis, MD, PGY4

Case of the week COW #6

CC: Vomiting for 2 days

HPI: 32 yo female with PMH of Romano Ward Syndrome s/p AICD, previous cardiac arrest, Atrial Fibrillation s/p Ablation, renal artery thrombosis on Coumadin and deafness presents to the Emegency Department (ED) via ALS after being found unresponsive at home and with ventricular tachycardia. Paramedics state they were called to the patient’s home for an “unconscious” person. They arrived to find the patient lethargic but arousable, cool, pale, and diaphoretic with BP 90/50. The patient soon became unresponsive and was in Ventricular tachycardia on the monitor. Patient underwent synchronized cardioversion with 150 Joules and converted to sinus tachycardia. Patient also received 150 mg Amiodarone bolus and 250 mL of Normal Saline IVF. Patient’s sister arrived to ED approximately 10 minutes after patient’s arrival and states that patient had multiple episodes of becoming unconscious at home earlier today. Denied recent illness, fever/chills, vomiting, diarrhea

Physical Exam:

BP 133/105,   HR 115 Sinus tachycardia,   RR 20,   SpO2 100% on 3 lpm NC,    Temp 97.0F

General: Lethargic but arousable to painful stimuli

HEENT: NCAT, pupils PERRLA, neck supple

Respiratory: Moderate respiratory distress with bibasilar rales, otherwise clear, no wheezing

Chest: AICD in left anterior chest

Cardiac: +S1/S2, no MRG, tachycardia, regular rhythm

Abdomen: soft NT ND

Neuro exam: arousable to painful stimuli, pt reading lips, answering appropriately

Extremities: weak thready pulses throughout, no edema

Skin: cool, pale, diaphoretic, no rashes, lacerations, or abrasions

 Labs:

CBC: Unremarkable     CMP: K 3.2   Glucose 595     BUN/Cr 17/1.62

AST/ALT 359/401       INR: 1.7             Troponin 0.122

ED Course: Approximately 5 minutes after arrival to the ED during the initial exam, patient became unresponsive and was noted to be in Ventricular Fibrillation on the monitor. CPR/ACLS was started and patient was intubated immediately. Her defibrillator appeared to be delivering shocks however there was no conversion of the rhythm. ACLS was performed for 30 minutes and pulses were regained multiple times. Patient was in V-fib, then PEA, then V-tach arrest. 100mg Lidocaine and 2 grams Magnesium were also given. Defibrillation with external pads ultimately led to Return of Spontaneous circulation. Bloody, frothy sputum was also noted from the ETT post-arrest. A right IJ central line was placed

EKG:

Pre-arrest: Atrial-paced with PACs. QTc 503      Post-arrest: Atrial tachycardia. QTc 609

DX: VFib & VTach arrest s/p ROSC in the setting of Congenital Long QT syndrome and AICD non-capture.

ED/Hospital course:  Post-ROSC EKG did not show STEMI. Post-ROSC Chest XR demonstrated diffused pulmonary edema. Patient was hypoxic with SpO2 in the 80s on 100% FiO2. Decreased tidal volume and increased PEEP improved oxygenation. Bedside cardiac Ultrasound showed global hypokinesis and no pericardial effusion. CT Head w/o contrast was normal. Amiodarone drip was started. Hypothermia protocol was initiated. The patient was admitted to the CCU. She did not code again while in the ED. Her AICD was interrogated and it showed she had been in sustained Vtach to which her AICD gave multiple shocks without capture. She was in the hospital for 11 days where she initially was improving and was therefore extubated after 7 days. Unfortunately she again went into Vtach arrest multiple times and then PEA arrest and was pronounced dead.

Pearls:

Congenital Long QT Syndrome (LQTS)

  • The major variant of congenital prolonged QT syndrome (there are 6 total). Affects estimated 1/2500 to 1/7000 people worldwide
  • Can have varying degrees of penetrance
  • Thought to account for ~3000/year of sudden deaths in children
  • May play a roll in prevalence of SIDS
  • Autosomal dominant inheritance, results in mutation of genes responsible for creating ion-channel proteins. The impaired transport of ions in cardiac tissue causes prolonged QT à impaired repolarization à ventricular arrhythmias (symptomatically syncope, seizure, cardiac arrest). May also present with AV blocks and bradydysrhythmias.
  • Romano-Ward is the more common, autosomal dominant (AD) form with purely cardiac phenotype.
  • The Jervell and Lange-Nielsen syndrome refers to the autosomal recessive (AR) phenotype of congenital LQTS that is associated with profound sensorineural hearing loss and a high risk for sudden death. MORE MALIGNANT CLINICAL COURSE. Our patient likely had this variation since she also had deafness.

Management and treatment

  • Beta- blockers = 1st line treatment in symptomatic patients. Blunts catecholaminergic response
  • Whenever syncopal episodes recur despite full-dose beta-blocking therapy, left cardiac sympathetic denervation (LCSD) should be considered. Cardiac pacing is only rarely indicated (e.g. in infants or young children with 2:1 atrioventricular block).
  • Implantable cardioverter defibrillators (ICDs) are always indicated after cardiac arrest, or when requested by the patient, and whenever syncope recurs despite beta-blockade and LCSD.
  • Potassium supplementation or spironolactone
  • Other antiarrhythmic therapy based on genotype

Risk Factors  for syncope or SCD

  • Congenital deafness
  • Hx/o syncope
  • Hx/o Ventricular arrhythmia
  • Family hx/o SCD
  • Female gender
  • QTc > 600ms
  • Medical non-compliance

Event triggers (vary depending on which genetic mutation)

  • Exercise-related
  • Auditory stimuli (alarm clock, telephone ringing!)
  • Acute emotional events
  • Catecholamine-induced
  • Pregnancy and up to 9 months post-partum
  • Onset of menopause

Post by Katrina D’Amore, DO.

 

Case of the Week (COW) #4

 

 

CC: Generalized Weakness

HPI: 55 y/o male presents complaining of 4 days of gradually worsening weakness in bilateral arms and legs. The patient also reports lower extremity pain one week ago, which resolved. Patient also complains of intermittent left sided back pain for the past couple of months that is exacerbated by walking. ROS: Positive for non-bloody diarrhea 2 weeks prior to presentation. Denies fever and vomiting at any time. No recent travels or sick contacts. Denies HA, dizziness, CP, SOB, or abdominal pain

Pertinent PE and Vitals: BP 172 / 82; Pulse 89; Resp 16; Temp 97.1; Pulse Ox 100% on R/A

GEN: Awake NAD.

EYES: PERRL, EOMI

NECK: supple, FROM, no meningismus

PULM: CTA in all fields, no tachypnea CARD: S1/S2 Normal. Normal rate.

NEURO: AAOX3. Lucid. Follows commands. CN 2-12 intact. No dysmetria bilaterally. No focal neurological deficits appreciated. Sensory intact. No drifts present in upper extremities. No dysarthria. Back: Mild painful ROM of lumbar region. No midline vertebral tenderness. No deformities. Deep tendon reflexes intact. 5/5 strength in all extremities.

Pertinent Labs (if any):

WBC 15.8 without any shift.

ED & Hospital Course: Patient was walked to evaluate gait in the setting of generalized weakness. She was able to ambulate with no ataxia. However, she was moving very slow and appeared globally weak. Patient stated this was not how she usually walks. A CT Head w/o Contrast followed by a lumbar puncture was then performed.

Lumbar Puncture (CSF Labs): RBC : 17,515   WBC: 21    Glucose: 103    Total Protein : 109

*LP was a traumatic 2nd attempt PLUS the samples were accidentally sent in a tube to the lab and dropped by the receiving lab tech. This contributed to the high RBC. Bonus Pearl – CSF tubes should always be hand delivered.

Working Diagnosis at time of Disposition : Guillain Barre Syndrome (GBS)

Neurology was consulted. Negative Inspiratory Force was performed with normal results. Patient was admitted to Neurology Floor with diagnosis of GBS. The patient improved after multiple doses of IVIG and was discharged from the hospital 1 week later with outpatient Neurology follow up

Pearls & Takeaways

Get a thorough history. Diarrhea is usually never important but in this case it was helpful in making the diagnosis. Ø Always walk your patients prior to discharge. Ø GBS is typically followed by a viral illness. Signs and symptoms include symmetric motor weakness that is usually ascending with little to no sensory involvement. Ø Progression of disease is over days to weeks. Ø Paralysis can ascend to the diaphragm. A Negative Inspiratory Force (NIF) test should be performed to evaluate the respiratory muscles. Intubate if FVC <15 mL/kg or negative inspiratory pressure < -25 cm H2O. Ø Albumin-cytological dissociation of CSF (high protein (>45) and low WBC count

 

Recent Pubs

We’ve had a bunch of publications in both peer-reviewed and non peer-reviewed sources over the past few weeks! Check them out when you get a chance:

Traficante and Kashani in the Journal of Clinical Toxicology talking about a Massive Calcium Channel Blocker OD

McGovern and D’Amore in Annals of Emergency Medicine talking about Peds EM Education 

D’Amore, McGovern and McNamee in ACEP Now talking about End-tidal for DKA and COPD

Pena, Mota and McGovern in AAEM/RSA Blog going over the elusive Porphyria diagnosis and management 

DeFranco and McGovern in ACOEP’s Fast Track talking about Isolated Bandemia