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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

ACEP Now Review on LVADs

Check out this excellent review on managing patients with LVADs from this month’s ACEP Now publication which was written by our own Dr. Yenisleidy Paez Perez, DO PGY-3 and one of our newly graduated residents, Dr. Terrance McGovern, DO. The article is entitled ‘How to Manage Emergency Department Patients with Left Ventricular Assist Devices.” Click the link below to be forwarded to the article.

http://www.acepnow.com/article/manage-emergency-department-patients-left-ventricular-assist-devices/

Breaking Bad News

One of the hardest roles of the emergency physician is giving bad news to a patient or their family members. Difficult topics that are often challenging for physicians to discuss include reporting the death of a family member or giving the diagnosis of a terminal illness. The optimal way to relay these topics is yet to be determined and each practitioner typically develops their own communication style. Some choose to deliver the message in a direct and succinct manner while others prefer a more drawn out and complete explanation. The Emergency Department provides a unique environment as there is often little time to develop any rapport with the patient and their family. This can make communicating these topics more challenging. In addition, breaking bad news involves more than just the verbal component of actually giving the bad news. It also requires the ability to respond effectively to patient’s and family’s emotional reactions and the dilemma of how to give hope when the situation is bleak.

Jurkovich et al studied the characteristics and methods of delivering bad news from the perspective of surviving family members. The chart below details the importance of various elements rated by respondents in the study. The attitude and clarity of the message delivered by the provider were deemed to be most important, while the attire of the provider had little importance to the respondents.

The duty of breaking bad news can be improved by understanding these characteristics and methods and then applying a step-wise method to effectively communicate and counsel patient’s and/or their families. In a recent Wednesday conference, Dr. Flannery, one of our core faculty attendings, introduced us to the SPIKES protocol for breaking bad news. The purpose of the protocol is to help the clinician fulfill the essential goals of gathering information, providing intelligible information, and supporting the patient or family by reducing their emotional impact and isolation. When we are informing our patients of an unfortunate diagnosis, the protocol also calls for collaborating in developing a strategy or treatment plan for the future. From the Emergency Department standpoint, this means guiding patients to the correct consultant for further workup and treatment options.

During our Wednesday conference, we broke into small groups and practiced situations that would be considered difficult to give bad news. The experience was positive and allowed us to give each other constructive criticism on ways to improve our approach to giving bad news. As a senior resident, I have unfortunately been involved in many of these situations throughout my residency. I have learned that despite the challenges involved in delivering bad news, there is also tremendous gratification in providing a therapeutic presence during a patient or family’s greatest need.

References:
1. Jurkovich et al. Giving bad news: the family perspective. J Trauma. May 2000
2. Baile W.F. et al. SPIKES – A Six-Step Protocol for Delivering Bad News: Application to the Patient with Cancer. The Oncologist. June 12, 2000.

EM Conference Pearls (8/2/17)

Pearl’s from Wed conference August 2nd 2017:

Agenda:
– Asthma/COPD: Baldino
– Sepsis Core Measures: Patel
– Pulmonary cases: Patel
– Medical student pearls (from Mike Taylor, one of our students)


Dr. Baldino: Asthma/COPD

-All that wheezes is not asthma (or COPD).
-Use diagnostics to rule out mimics such as pneumonia or ptx.
-Get the CXR in COPD exacerbation, not routinely in simple asthma exacerbation.
-Good evidence and NNT’s for benefit of ipratropium, systemic steroids, magnesium,  and BiPAP.
-Intubation last resort for asthma.  Remember to adjust I to E ratio on vent.
-Steroids at discharge for asthma/COPD.  Antibiotics at discharge for COPD.
-Discharge with a plan! (and a spacer)


Dr. Patel: Sepsis Core Measures

-Sepsis core measures are from CMS, not from SSC guidelines or Sepsis 3.0. They are not necessarily rooted in great evidence, but we have to follow them!
-Remember the 3 and 6 hour severe sepsis and septic shock bundles. Timing is based on presentation time (when chart displays severe sepsis, septic shock), not door time.  To make your life easy, just use door time to meet the metrics.
-The focused exam for septic shock can now just be documented with one statement, which is in Medhost.  Make sure to click that.
-Fluids from the field count (as your 30 cc/kg), as long as it is given as a bolus and documented on the chart.
-Antibiotic choice and timing both looked at for core measures. For choice, best to go with a monotherapy agent first to meet the metric.


Dr. Patel: Pulmonary Cases

-The term HCAP is not in the newest pneumonia guidelines from 2016.
-Treat HCAP like CAP unless the patient is going to the MICU.  If going to the MICU, cover for MRSA and Pseudomonas.

Hemoptysis:

-Minor hemoptysis (streaks in the sputum)–d/c unless CXR abnormal
-Moderate hemoptysis (frank hemoptysis)—admit for further work up and obs
-Massive hemoptysis (hemoptysis interfering with respirations)–intubate and consult pulmonary (for bronch) and IR (for possible bronchial artery embolization). If there is a suspicion of a bronchovesicular fistula or other arterial fistula, CT surgery may also need to be on board.


Medical Student Pearls

One of our current medical student’s Mike Taylor put together some info on questions that were raised in conference:

Intentional “L Main Bronchus Intubation:” (for hemoptysis)

Take Home Points from 1995 Anesthesiology Case Report:
 -Can use a double lumen ET tube with a endobronchial cuff
 -The inflated endobronchial cuff can tamponade the hemorrhaging R lung and occlude airflow into it. This allows only the L lung to be effectively intubated and the provider not have to be tasked with putting the tube in the L main bronchus
 Reference: http://anesthesiology.pubs.asahq.org/article.aspx?articleid=1949905

Rate Control for MAT:

Take home points from Uptodate
 -Treat underlying cause
 -Maintain phos and mag levels
 -Can use CCBs or beta blockers for rate control in symptomatic pts
 Reference (requires access to UpToDate): https://www.uptodate.com/contents/multifocal-atrial-tachycardia

Bandemia Cut Offs:

Take home points from 2012 Retrospective Cohort Study:
 -With normal white counts, pts with bandemia of at least 11% had higher in hospital mortality
 -So 11% or higher could use as a cut off for admission, more aggressive treatment, etc.
 Reference: https://www.ncbi.nlm.nih.gov/pubmed/22939096

Special thanks to Chief Dan Poor PGY-4 for organizing this week’s Conference Pearls and for Mike Taylor MS-IV for his Medical Student Pearls

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.

 

EM Conference Pearls (7/26/17)

Pediatrics Trauma Radiology Review (Dr. Flannery) – When in doubt splint

  • Salter Harris: Take home memorize classification and management
    • SH1: Clinical Dx – Splint and f/u
    • SH2: Splint and f/u, most common
    • SH3: Ortho consult
    • SH4: OR
    • SH5: OR
  • Supracondylar Fx : Take home: Type II/III requires OR
    • SC Fx type I: Look for posterior/anterior fat pads
      • Tx: Posterior splint, f/u ortho
    • SC Fx type II: Splint and Ortho consult
    • SC Fx type III: OR
  • Misc Fx
    • Greenstick fracture
    • Toddle Fx: Tx with posterior splint and out pt f/u (For non displaced)
      • When in doubt splint and f/u ortho
    • Buckle Fx: Volar splint
  • SCFE
    • Klein’s line should intersect the femoral head
    • Can be bilateral
  • SCIWORA
    • Always recheck spinal (ROM/focal findings) after a negative CT cervical

Trauma Board Review (Dr. Patel)

  • Hip/Femur fracture
    • Most are operative, recognize they are sick patients.
    • Fermoral head fx, neck fx, trochanteric (Inter/sub)
    • Pain control, Ortho consult, Most cases OR
  • Tibial plateau fracture
    • Maybe radiographically occult
    • Patella alta
  • Maisonneuve fracture
    • Proximal fibular fx + medial malleolar fracture ( or disruption of deltoid ligatment, IO membrane syndesmosis)
  • Lisfranc fracture
    • Tarso-metatarsal fracture-dislocation
    • Mal-alignment Metatarsals with cuneiforms.
  • Calcaneal fractures
    • 10% associated lumbar fractures
    • Boehler’s angle <20
  • Jones fracture: High rate on non-union
  • Pseudojones fractures: Bulky dressing and pain control
  • Hip dislocations
    • MC: Posterior dislocation (90%)
    • High rate of AVN femoral head, peroneal nerve dysfx, sciatic nerve injuries)
  • Knee dislocation
    • 50% will spontaneous reduce!!!
    • Need a good Neurovascular evaluation
    • ABI < 0.9
    • Vascular surgery/ortho eval à Arteriography or CT Angio
  • Ankle Sprain:
    • Ottawa ankle rule
  • Scapular fracture
    • Associated with concomitant injuries such as internal thoracic injuries
  • Clavicle fracture
    • Op: Open fx, unstable floating shoulder, NV compromise.
  • Radial head fx
  • Monteggia fracture
  • Galeazzi fracture
  • Scaphoid fx – Thumb spica, risk of non-union and AVN
  • Bannette’s fx – Thumb spica,
  • Rolando fx
  • Posterior shoulder dislocation: Light bulb / Drum stick appearance
  • Compartment syndrome
    • 6P’s. Money is on pain and paresthesia.
  • High pressure injury injection injury
    • Limb threatening
    • Surgical emergency

Trauma in pregnancy (Dr. Kashani)

  • Prepare for difficult airway
  • Be aware of Hemodynamic changes
  • Rh sensitization
  • >20 weeks with abdominal trauma monitor for 4 hrs
  • Peri mortem C-section best outcome if performed within 4 – 5 minutes of CPR 

Thanks to Chief Mike Hong, DO PGY-4 for writing up today’s CONFERENCE PEARLS

Case of the Week (COW) #5

CC: Vomiting for 2 days

HPI: 47 year old female with PMHX of HTN and ETOH abuse presents with abdominal pain. Patient states that beginning two days ago she was woken up from her sleep with sudden onset non-bloody vomiting as well as epigastric pain which radiates to the back and is sharp in nature. She admits to over 15 episodes of vomiting. She is also having cramping of her feet bilaterally. She admits to daily ETOH use, and states her last intake was two days ago, denies illicit drug use. Denies taking any medication prior to arrival. Denies fever, chills, chest pain, SOB or dysuria. Denies recent travel or sick contacts. PMHX/PSHX: none Meds: none Allergies: none

Physical Exam: Vitals: BP 150/96 P 98 RR 16 O2 sat 98% RA General: Awake, alert, anxious Cardiac: Regular rate, no murmurs Lungs: CTAB, no rales, no rhonchi, no wheezing Abd: soft, non-distended, Mild tenderness to palpation epigastric area

Labs: Magnesium- 1.3 Potassium- 3.5 Troponin- 0.017

EKG:

DX: Prolonged QT interval with non-sustained polymorphic Ventricular tachycardia secondary to Hypomagnesemia

ED/Hospital course:  Upon arrival to ED patient had Epigastric pain with vomiting, Patient started on IVF and received Pepcid and Zofran. EKG at this time showed QT prolongation and patient found to have magnesium of 1.3. While waiting for magnesium, she started to have short runs of polymorphic ventricular tachycardia and during these times she complained of chest tightness. After 2 grams of magnesium patients repeat EKG showed normal QT and runs of ventricular tachycardia stopped. She received another 2mg of Magnesium and 40 mEq of Potassium Chloride. Patient was then admitted to Telemetry floor. Patient observed for 24hours and discharged to home with follow up with a cardiologist and Norvasc 5 mg 1tab PO daily, Losartan 100 mg 1tab PO daily, Ranitidine 150mg 1tab PO BI.

Pearls:

  • An abnormally prolonged QTc, especially >500 is associated with an increased risk of ventricular arrhythmias, Torsade’s de Pointes
  • Prolonged QT with prolonged T wave is due to: HypoK, HypoMg, Medications, Elevated ICP, Cardiac ischemia, Congenital.
  • Prolonged QT with prolonged ST-segment is due to: HypoCa, Hypothermia.
  • If EKG reveals long QT start by reviewing drug history and checking electrolytes. Stop any offending agents. Suppress early after depolarization with IV magnesium sulfate and keep potassium >4.5meq/L.
  • If non responsive to magnesium, may consider cardiac pacing and rarely isoproterenol infusion. Acceleration of the heart rate may produce suppression of arrhythmias, with a reduction in the QT interval.
  • Unstable patients should undergo non-synchronized electrical defibrillation.

Post by: Dr. Kerri Clayton, 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

 

Steroids for SJS and TEN?

Here’s a quick hit summary of the evidence regarding the use of systemic corticosteroids in the treatment of SJS/TEN

  • Small amount of evidence, NO RCT to date
  • Small retrospective study (n=30, groups comparable) in 1984 from a burn center found survival benefit (66% versus 33% survival) in NOT giving steroids. Also found decreased complications such as Candida sepsis & esophageal ulcer in patients who did NOT receive steroids.
  • Cohort of ~500 patients from RegiSCAR (International Registry of Severe Cutaneous Adverse Reactions to Drugs)
    • No statistical diff in Hazard Ratio among treatment groups (supportive care vs. corticosteroids vs. IVIG).
  • Systematic Review of literature from 2001-2009 (only used Pubmed, not great)
    • Pooled analysis demonstrated no statistically significant difference in Mortality Ratio among groups (supportive care vs. corticosteroids vs. IVIG).
    • B.: i2 statistic not reported but authors mention no problematic heterogeneity.
  • Very small study (n=12 over 10 years) demonstrated potential benefit to early pulse-dose IV steroids (1.5 mg/kg/day dexamethasone for 3 days) in the form of (1) disease halt at 3 days (2) 1 actual death versus 4 predicted deaths.
  • Interestingly, a case-control study (case n=92; control n=381) demonstrated that pre-existing chronic steroid use delayed onset of SJS/TEN in patients using high-risk drugs by 7 days but also prolonged disease course by 2 days.

Bottom line

  • Systemic corticosteroids have not been shown to consistently correlate or provide a survival benefit in patients with SJS/TEN.
  • Paucity of evidence may show benefit to pulse-dose IV steroids such as are used in the treatment of autoimmune diseases such as pemphigus vulgaris.
  • Supportive care is the standard of care.

Post by: Dr. Katrina D’Amore DO, MPH

Case of the Week #3 – Why my flap sunken?

CC: Altered mental status and frequent falls

HPI: 55 y/o male presents from rehab with altered mental status (lethargy and agitation) and frequent falls for the past 2 days. Patient has a PMHx of large traumatic subdural hematoma 9 months ago treated with a craniectomy, seizure disorder, and HTN. Patient complains of headache and is a difficult historian. Pt does answer some questions appropriately.

Pertinent PE and Vitals: BP 121/81 HR 98 RR 16 Temp 98.1 100% on RA

GCS=14 and in no acute distress

L pupil dilated at 4 mm and nonreactive. R pupil 1 mm and reactive.

Pt. with moderate R arm and leg weakness (patient has baseline weakness but this is worse).

Pertinent Labs (if any): Unremarkable workup

Imaging

Working Diagnosis at time of Disposition Sinking Skin Flap Syndrome (also known as Syndrome of the Trephined) with possible Paradoxical Herniation

ED & Hospital Course Patient was admitted and received neurology and neurosurgical consultations. Medications were adjusted to control agitation. It is questionable per the consultants if his symptoms were due to paradoxical brain herniation. Plan is for an outpatient cranioplasty.

Pearls & Takeaways

  • Sinking skin flap syndrome is a delayed complication of a decompressive craniectomy. As the herniated brain tissue recedes, the skin flap from the surgical site can become sunken.
  • Symptoms include headaches, dizziness, seizure, and mood changes.
  •  Symptoms worsen when is head elevated vs reclined; treatment option is cranioplasty. Symptoms are much worse in an upright posture.
  • If atmospheric pressure exceeds intracranial pressure, patients can get paradoxical herniation and midline shift. This is more of an emergency and symptoms include focal deficits, pupillary changes, and alterations in consciousness.
  •  Paradoxical herniation is a state of low intracranial pressures; therefore traditional measures to treat midline shift and ICP will worsen the condition such as mannitol, hyperventilation, etc.
  • Treatment of sunken skin flap with paradoxical herniation is to elevate the intracranial pressure, including Trendelenburg position, hydration, and clamping of any CSF drains. Definitive treatment is cranioplasty.