Varicella in Pregnancy

Varicella-zoster virus

Varicella (chickenpox) is a highly contagious disease caused by primary infection with varicella-zoster virus (VZV)2 and may cause maternal mortality or serious morbidity

> Reactivation of latent infection, usually many years after the primary infection, may result in herpes zoster (shingles), a painful vesicular eruption in the distribution of sensory nerve roots2

> Both varicella (chickenpox) and zoster (shingles) are notifiable diseases. Notification must be made to the Communicable Disease Control Branch of SA Health as soon as possible and at least within three days of suspicion of diagnosis, by telephone or post.


Route of transmission

> Infection with chickenpox is transmitted through airborne / respiratory droplets and direct contact with vesicle fluid


Incubation period

> 10 to 21 days (may be up to 35 days in contacts given high titre zoster immunoglobulin, ZIG)


 Period of infectivity

> 48 hours before the onset of rash until crusting of all lesions (usually day 6 of rash)

> Infectious period may be prolonged in people with impaired immunity


Infection Control

> Non-immune staff should not care for the woman / baby infected with chickenpox

> Varicella (and herpes zoster) vesicles contain large numbers of virus particles. Ensure appropriate transmission based (standard, contact and airborne) precautions including:

> A negative pressure room with door shut (chickenpox and disseminated shingles)

> Immune staff in attendance

> Gloves, gown

> All dressing materials should be treated as medical waste

> Chickenpox and disseminated shingles use standard, contact and airborne precautions

> Localized shingles use contact precautions (only immune staff in attendance, single room, gloves, gown)

> In herpes zoster (shingles), transmission of infection usually requires contact with vesicle fluid; however, there is also evidence of respiratory spread. Localized shingles requires standard and contact precautions (not airborne precautions)


Susceptibility to varicella

> Women and babies susceptible to infection with VZV (may be severe or life-threatening) include:

> No history of varicella (chickenpox or shingles)

> Seronegative for varicella antibodies (VZV-IgG negative)

> No documented evidence of varicella vaccination 


Significant Exposure

> For the purpose of infection control and prophylaxis, significant exposure of a susceptible woman who is pregnant to varicella includes:

> Living in the same household as a person with active varicella or herpes zoster

OR

> Direct face to face contact with a person with varicella or herpes zoster for at least 5 minutes

OR

> Being in the same room for at least 1 hour

> Chickenpox cases are infectious from 2 days before rash until lesions crusted


Management of maternal exposure to varicella-zoster virus

History of previous chickenpox

> No action required

No or uncertain history of chickenpox

> Obtain serology for antibody status (VZV-IgG) (if practicable)

> ZIG if required should be given within 96 hours. Testing should only be done if ZIG would still be able to be given, if required, within this window


Within 96 hours from exposure

> Zoster immunoglobulin (ZIG) should be given to all seronegative women within 96 hours (see adult dose under ZIG dosage below)

> However there may be some limited effect out to as late as 10 days post exposure

> Advise to seek medical care immediately if chickenpox develops


More than 96 hours following exposure

> Oral aciclovir or valaciclovir (see dosage below) should be considered for women:

> In the second half of pregnancy

> With a history of an underlying lung disease

> Who are immuno-compromised

> Who are smokers

Note: Advise women to seek medical care immediately if chickenpox develops


Management of varicella-zoster in pregnancy

Less than 24 hours since appearance of rash

> Oral aciclovir 800 mg 5 times a day for 7 days OR oral valaciclovir 1 g three times a day for 7 days]

> Monitor at home

More than 24 hours since onset of rash

> No oral aciclovir / valaciclovir and monitor at home if:

> No underlying lung disease

> Not immunocompromised

> Non-smoker

> Monitor in hospital if any of the above risk factors

> Offer appropriate fetal medicine counselling


Complications

> Advise to seek medical attention for the following complications:

> Respiratory symptoms

> Haemorrhagic rash or bleeding

> New pocks developing after 6 days

> Persistent fever

> 6 days

> Neurological symptoms

> Give aciclovir 10 mg / kg every 8 hours for 7 to 10 days (IV followed by oral [see dosage below]) and administer supportive therapy

Consider caesarean section if:

> Signs of significant fetal compromise

> Evidence of maternal respiratory failure exacerbated by advanced


Risk of fetal varicella syndrome (FVS) after maternal VZV

Timing of maternal infection:

>Less than 12 weeks gestation-  0.55%

>12-28 weeks gestation- 1.4%

>More than 28 weeks gestation- No cases of FVS reported


Refer to maternal fetal specialist for prenatal diagnosis and counselling

> Detailed fetal ultrasound for anomalies is recommended at least five weeks after primary infection

> Repeat ultrasounds until delivery. If abnormal may consider fetal MRI

> VZV fetal serology is unhelpful

> Amniocentesis not routinely advised if ultrasound normal, because risks of FVS low but negative VZV PCR may be reassuring


Management of infants exposed to maternal varicella zoster

Maternal chickenpox > 7 days before delivery

> No zoster immunoglobulin (ZIG) required

> No isolation required

> Encourage breastfeeding

> No other interventions even if baby has chickenpox at or very soon after birth unless preterm < 28 weeks gestation or low birth weight < 1,000 g > Very preterm infants (≤ 28 weeks gestation) born with chickenpox should receive intravenous aciclovir 20 mg / kg / dose every 8 hours as a slow infusion (1-2 hours)

 

Thank you to Christine DeFranco PGY-4 EM Resident for this review of VZV in pregnancy!

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

Case of the week COW #11

CC: Can’t see for 2 months

HPI: 10 year old male with no significant past medical history presents to the Emergency Dept. complaining of painless vision loss in the right eye for 2-3 months. Patient states he has been having difficulty seeing out of his right eye for 2 months despite changing positions in class, taking on and off his glasses and using eye drops. Vision loss has become progressively worse and now he can only distinguish whether lights are on or off. Mother states she did not do anything about this problem because she thought he was kidding and she didn’t’ have money or health insurance to seek medical attention. Patient has been wearing Bifocals for myopia since the age of 6, with corrective vision lenses at age 5. Denies associated trauma. Patient moved to the U.S from Nigeria 5 years ago. He lives with his mother and sister. Vaccines are up to date.

PMH/Birth History: NSVD at 39 weeks, 1 day. Birth took place in Nigeria and mother denies any infection during or after pregnancy. Following birth, patient did not have any complications such as pneumonia, eye infections or rashes.

Social History: Denies drug abuse. Denies exposure to chemicals. Denies contact with dirt, dogs or pigs. Patient did not live on a farm. As per mother, there was no domestic abuse in the family

PHYSICAL EXAM 

Vitals within normal Limits

General: In no acute distress, appears stated age.

Head: Atraumatic, normocephalic. No deformities.

Eye: Right (OD): pupil non-reactive and remains white in color and approximately 2mm. Afferent reflex is present CN II; efferent reflex is absent. There is a complete visual field defect on the right side. Visual Acuity: Can only distinguish between light and dark. Unable to count fingers at any distance or visual objects even in motion. On Fundoscopic exam, the fundus is gray/dull, with no retina visualized. ALL CONSISTENT WITH LEUKOCORIA

Respiratory: Lungs CTA bilaterally.

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

Abdomen: soft NT ND

Neuro exam: CN III –XII intact. No motor, sensory, vibratory, temperature or pain deficits. Gait is steady and normal, without any difficulty. Cerebellar function intact, no dysmetria bilaterally.

Extremities: Full ROM in all extremities. 5/5 strength in all extremities

Skin: No edema, rashes, lacerations, or abrasions. Skin is warm, pink, moist and intact.

LABS

CBC: Unremarkable with        WBC: 7.1     Hemoglobin: 13.5       Platelets 211.

CMP: WNL       CRP and ESR = WNL

Tonometry: IOP approx. 17-18 x 3 times

Wood lamp: No corneal abrasion, dendritic ulcers, lesions or depositions OU

Bedside U/S: Completed within 10 minutes of arrival ( Only Right eye is shown)

Working Differential Diagnosis:

  • Retinoblastoma
  • Intraocular Mass
  • Cataract
  • Syphilis
  • Parasitic infiltrate
  • Posterior Infarct

CT Max/Facial w/o Contrast to rule out intracranial pathologies: Negative for masses; positive for homologous material in the right lobe. MRI was recommended for further evaluation

ED/Hospital course:  Ophthalmologist on call was consulted, and said there was nothing more to do. Unfortunately because his vision loss was > 2 months, no life saving surgery was imminent as the retina was already detached causing ischemia to the rods and cones within it.  Patient was to follow up in ophthalmology clinic the following day. Patient’s Pediatrician was also contacted and he referred the patient to a Pediatric Ophthalmic specialist in University Hospital, with a scheduled appointment within the same week. Approximately one week ago, patient underwent surgery and the leading diagnosis was Toxocariasis as per MRI. Biopsy results to follow. As per mother, the patient is still without vision in his right eye.

LEUKOCORIA, UNILATERAL, 10 YO MALE DIFFERENTIALS

Coats disease is an exudative retinal vascular disorder characterized by retinal telangiectasias and subretinal exudation leading to serous retinal detachment. Presenting complaints include decreased visual acuity, strabismus, or leukocoria. The leukocoria in Coats disease is generally more yellow than white due to the presence of subretinal lipid. Coats disease is almost always unilateral and the vast majority of cases occur in boys. The majority of cases presenting with leukocoria are diagnosed between five and nine years of age, which is older than the majority of retinoblastoma patients.

Clinical examination: subretinal lipid and abnormal telangiectatic vessels US: demonstrates complete retinal detachment with massive subretinal lipid. Intraocular calcification is almost never present in Coats disease, another feature that helps to distinguish it from retinoblastoma.

The retinal photograph depicts bullous retinal detachment in the Left eye of a 1-year-old child with Coat’s disease simulating retinoblastoma. The magnetic resonance images confirm the diagnosis of retinal detachment and exclude retinoblastoma because of the absence of intraocular mass.

 

Toxocariasis — Toxocariasis, or visceral larva migrans, is an infection caused by the dog ascarid Toxocara canis or, less commonly, the cat ascarid Toxocara catis. There may be a history of living in an underdeveloped country, and exposure to dirt or undomesticated dogs. It occurs most commonly in children one to five years of age. Common presenting signs of toxocariasis are strabismus and poor vision. The ocular lesion is caused by the inflammatory response to the second-stage larva, which may localize in the one eye or both eyes

Chorioretinitis characteristic of toxoplasmosis. A pigmented scar is seen with an adjacent area of active chorioretinitis. The diagnosis of toxoplasmosis is based primarily on the appearance of the chorioretinal lesion rather than serologic studies. Courtesy of James T Rosenbaum, MD.

 Retinopathy of prematurity (ROP) — developmental vascular proliferative disorder that occurs in the incompletely vascularized retina of preterm infants and can lead to retinal detachment and permanent blindness. The most important risk factor for developing ROP is prematurity. There needs to be a history of prematurity, and/or mother mentions patient was on O2 for awhile.

Vitreous hemorrhage — Vitreous hemorrhage causes leukocoria when there is extensive organization of the blood into a clot before degradation. With time, the reddish hue of the blood is lost and the hemorrhage transforms into “whitish debris.” Etiology includes advanced ROP,  trauma (MOST common), leukemia or hemorrhagic disease of newborn

Retinablastoma– Retinoblastoma is the most common primary intraocular malignancy of childhood and accounts for 10 to 15 percent of cancers that occur within the first year of life. Retinoblastoma typically presents as leukocoria in a child under the age of two years. Untreated retinoblastoma is a deadly disease; however, with advances in treatment, survival in the contemporary era is >95 percent. Most common age group is birth- 2 years. Very uncommon in children over the age of 5, although 1-2 case reports have occurred in 18 year old males

 Pearls & Takeaways:

  • Leukocoria Requires further investigation – On exam, History and Diagnostic
  • The use of tonometry, fundoscopy and ocular ultrasound are an easy, noninvasive way to add to your ophthalmic exam. Slit-lamp exam could have been considered here
  • Ocular ultrasound is really cool and fun!
  • Advocate for your patients!  Make sure they have proper follow up with a specialist
  • If any abuse or neglect is suspected, be sure to turn on “Sherlock Holmes” senses and investigate.

 

Case presented by Dr. Sarah Bolan

 

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

Case of the Week COW #9

CC: “Post Surgical Pain” ; Abdominal pain

HPI: 8 year old Male with PMH of Sickle Cell Disease (HbSC), Post-opt Day 10 for laparoscopic splenectomy for recurrent sequestration crises presents to the Emergency Department (ED) complaining of abdominal pain x 2 days. The pain is described as diffuse and worse in the RUQ. Denies exacerbating or relieving factors. Pain is associated with constipation; last Bowel movement was 7 days prior. Mother states he did have one small BM shortly after surgery. He is tolerating PO without difficulty and does report flatus. She has been giving him Oxycodone 2mg every 6 hours for pain with minimal relief. Upon review of systems, patient reports feeling hot for the past 2 days but without a recorded temperature at home. Additionally, patient complained of a non-productive cough for 2 days and has been “breathing fast” for the same duration of time.

Physical Exam:

BP 112/74    HR 129     RR 24   SpO2 93% on RA   Temp 99.9F      26.3 kg

Constitutional: Well developed, well-nourished child who is awake, alert and in moderate distress due to pain and feeling hot; making tears

HEENT: NCAT, pupils PERRLA, neck supple

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

Chest/Axilla: Normal symmetrical motion, no tenderness

Cardiac: +S1/S2, RRR, no MRG,

Abdomen: Scars noted over splenectomy site which are clean dry and intact, healing well without discharge; Diminished Bowel Sounds in all quadrants; soft, non-distended with mild tenderness in all 4 quadrants. No rebound or guarding.

Neuro exam: AAO x 3. No deficits

Extremities: no edema, no tenderness or swelling

Pertinent Labs:

CBC: 42.1

HBG: 12.5

HCT: 36.9

Platelets: 721

Seg: 79

Lymphs: 9

Mono: 12

Reticulocyte count: reported as normal

Pertinent Imaging and other tests:

Working Diagnosis:

Generalized abdominal pains

Elevated WBC

Abscess of spleen

ED/Hospital course:

Surgery was consulted in the ED and they believed the pain was not related to the surgery. Abdominal U/S performed at their request showed “s/p splenectomy with gallbladder sludge.” Patient had moderate improvement in his pain after Toradol 15mg IV and Morphine 2mg IV were given in the ED. Patient also received Rocephin 75mg/kg and NS 500mL IV Fluid bolus for presumed infection with the leukocytosis of 42.1.

The patient was admitted to Pediatric Step down for further evaluation and management. Overnight, he remained comfortable with stable vital signs, afebrile and saturating 94-96% on 2L NC. On Hospital day #1, patient became febrile to 103F and continued to deny chest pain, SOB or difficulty breathing. The cough remained unchanged since admission. A repeat Chest X-Ray was obtained and the patient was diagnosed with Acute Chest Syndrome and received exchange transfusion the same day (Refer to Image below) Additionally, he received Ceftriaxone, Azithromycin, Nitric oxide and IVF at ¾ maintenance ate throughout hospitalization. On Hospital Day #4 he was discharged home.

Pearls:

Acute Chest Syndrome

  • It is the leading cause of death in patients with SCD in the United States
  • Most often occurs as a single episode, but patients may have multiple attacks resulting in chronic lung disease
  • Multiple Etiologies
    • Pulmonary infections
    • Iatrogenic cause including aggressive hydration for sickle cell painful crisis ——à causing Pulmonary Edema
    • Opioid use —–à Decreased inspiratory effort——à Atelectasis
  • New infiltrate identified on Chest X-Ray with at least one of the following: Fever, cough, wheezing, tachypnea, chest pain, hypoxemia.
  • Radiographic changes often lag behind clinical features so initially the Chest X-Ray may actually be normal (as in our case)
  • Treatment
    • Supportive: Early Supplemental Oxygen, analgesics and IV Hydration up to 1.5x maintenance rate
    • Antibiotic irrespective of cultures
    • Transfusion is believed to be lifesaving; recommendations based on empirical observations and not on firm evidence
    • Exchange transfusion seems to be more advantageous, especially in patients with a hemoglobin of > 9.0
      • It decreases the concentration of sickled hemoglobin with little iron gain
    • Inhaled Nitrous Oxide is beneficial
      • Due to its vasodilatory effects –à improved ventilation/perfusion in damaged lung tissue
      • Reduced RBC and leukocyte adhesion to endothelial cells, therefore affecting disease progression
    • Hydroxyurea reduces occurrence of acute chest syndrome

Case Presented by Greg Cassidy, MD

 

References:

  1. Stapczynski, J. S., & Tintinalli, J. E. (2016). Tintinalli’s emergency medicine: A comprehensive study guide (8th ed.). New York, N.Y.: McGraw-Hill Education LLC..

 

Case of the Week COW #8

CC: Altered Mental Status

HPI: 50 -year-old Male with PMH of HIV, CVA and Meningitis presents to the Emergency Department (ED) for altered mental status. As per the patient’s girlfriend at bedside, the patient woke up confused and was not making any sense when he spoke. He even urinated on the floor but does not remember doing so. Patient had been complaining of back pain, testicular pain and leg pain for the past couple of weeks and had been evaluated for it in the ED. Patient also states he is currently taking “something for his HIV” but is unclear what his last CD4+ count was. Denies sick contacts. The rest of the review of systems was limited by confusion, but denied vomiting, diarrhea, abdominal pain, or any other complaints.

Physical Exam:

BP 128/78   HR 129     RR 14   SpO2 96% on RA   Temp 102.4F

Constitutional: Diaphoretic, confused and intermittently following commands.

HEENT: NCAT, pupils PERRLA, neck supple

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

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

Abdomen: soft, mildly distended with mild tenderness in RUQ and LUQ.  Was not able to appreciate any focal masses . No rebound or guarding

Neuro exam: Not oriented to time or situation, No focal deficits, moving all four extremities. Unable to complete a more detailed exam as patient remained confused.

Extremities: no edema, no tenderness or swelling

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

Pertinent Labs:

(Per Sorian Inpatient) CD4 = 120 on June 2017

Sepsis workup summary (normal if not reported):

  • Trop 0.045ng/ml
  • Sodium 126
  • WBC 5.8       Chloride 93
  • RBC 3.72      CO2 19
  • HBG 10.3      Glucose 116
  • HCT 30.3       BUN 89
  • Platelets 108
  • Cr 2.90 (↑ from baseline)
  • Bands 27        Total Bili 4.5
  • Lymphs 3       Total Protein 5.7
  • Monos 2         Albumin 2.6
  • Lymphocytes 0.2
  • Alk Phos 346   Monocytes 0.1
  • AST 143
  • ALT 58
  • Lactic acid 3.4
  • U/A negative
  • CSF negative

Pertinent Imaging and other tests:

EKG remarkable for sinus tachycardia, left axis deviation, and an old RBBB

CT head w/o contrast remarkable for only mild frontal volume loss

Chest XR – unremarkable

RUQ Bedside US and then official US completed and showed:

Working Diagnosis:

Hepatic hydatid cysts from Echinococcus tape worm

Hepatic abscesses

Metastatic cancer

Multiple biliary hamartomas

Polycystic liver disease

Caroli Disease

ED/Hospital course:

In the ED, the patient received IV Fluid boluses of NS 30mg/kg and one 1000mL of NS along with Tylenol, Vancomycin and Zosyn. The patient was admitted to Infectious Disease service. Throughout the hospital stay, CT Scan of abdomen and pelvis w/o contrast (due to AKI) was remarkable for infiltrated liver, splenic lesions, and destructive lesions of the bilateral iliac wings and L5 with pathologic fracture of the posterior right rib, which may be due to metastatic disease. The underlying etiology is uncertain. Without contrast, it was not certain if there was underlying macronodular cirrhosis. There was also associated ascites. Initial blood cultures from the ED grew Salmonella species. The patient was initially admitted the medical floor but was transferred to the Medical ICU on day 7 of hospitalization for increased lethargy and worsening lactic acidosis, transaminitis, and AKI. He later went into multisystem organ failure and was intubated thereafter. His code status was also changed to DNR/DNI. The patient unfortunately expired before endoscopy, colonoscopy, and biopsy could be performed.

Official Ultrasound read – Findings consistent with metastatic disease to the liver.

Pearls:

Hepatic Hydatid infection

  • Caused by Echinococcus granulosus or Echinococcus multilocularis
  • granulosus – Endemic in North America & Australia with dogs & wolves main as main host
  • multilocularis – Found in Northern Hemisphere with red fox, dogs, & cats as main host
  • Ultrasound would show a multiseptate cyst with daughter cysts
  • X-ray would show calcified rings
  • CT Abd/Pelvis may show the water-lily sign, which occurs when the endocystic membrane becomes detached, resulting in floating membranes within the pericyst, which mimics the appearance of a water lily ( Refer to Figure 1).

Figure 1. A detached membrane within the contents of the cyst, known as the water-lily sign

Pearls

Hepatic Hydatid infection

  • Infection may be asymptomatic for many years, with a long latent period (up to 50 years of age!)
  • Albendazole for confirmed infection
  • Reserve antibiotics for those in which diagnosis is uncertain due to risk of anaphylaxis
  • Most cases in U.S. occur in immigrants from endemic countries (South America, Middle East, eastern Mediterranean, sub Saharan, African, West China, former Soviet Union)
  • Confirmed cases in U.S. are rare

Patients with HIV

  • Always ask for CD4+ count and if they are on medications for their HIV/AIDs
  • Have a low threshold for doing an aggressive workup for these individuals, especially if poor follow up
  • HIV is a risk factor for Salmonella bacteremia
    • Other risk factors include any immunosuppressed state, liver disease, hemoglobinopathies (decreased splenic function)
    • Most salmonella bacteremia can have a preceding diarrheal illness
    • Major complication is endovascular infection
    • Treatment is IV fluoroquinolones or 3rd generation cephalosporin

Case presented by Jessica Williams, MD, PGY1

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) #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

Who got that stank?

Neutralizing ED Stank

Patients that stink can really hinder an ED. It creates a difficult work environment for ED staff and makes nearby patients, families and visitors uncomfortable. Here are a few ways I came across to neutralize the stank:

Nebulize stuff in the patients room

 

  • Nebulized OJ (works well for malodorous feet)
  • Nebulized coffee (works well for melena)
  • Will partially mask the smell
  • Works better in enclosed rooms w doors

 

image1.JPG

For when you have to remove shoes & socks (malodorous feet):

1. Maalox booties

  • Place feet in large trauma booties
  • Fill w Maalox and allow to soak

2. Towel Soaks

  • Mix warm water, betadine, peroxide and hibiclens (chlorhexidine) in basin
  • Soak towels in solution then wrap feet
  • Wrap feet and towel w blue pad
  • Allow 15 minute soak

3. Topical sodium bicarb

  • Use 1 amp per foot, apply topically
  • Neutralizes most odors
Post by: Dr. David Traficante (@davetraf)
References: