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Acute Chest Syndrome
Gabriela Rivera Camacho, MD & Mark Kastner, MD
Departments of Emergency Medicine & Hematology
Carolinas Medical Center & Levine Cancer Institute
Charlotte, North Carolina
Michael Gibbs, MD
Chest X-Ray Mastery Project™
Lead Editor
Ify Osunkwo, MD
Padmaja Veeramreddy, MD
Guest Editors
Disclosures
 This ongoing chest X-ray interpretation series is proudly sponsored by the
Emergency Medicine Residency Program at Carolinas Medical Center.
 The goal is to promote widespread mastery of CXR interpretation.
 There is no personal health information [PHI] within, and all ages have
been changed to protect patient confidentiality.
Process
• Many of the provided clinical cases and presentations are then shared with
all contributors on our departmental educational website.
• Contributors from many Carolinas Medical Center departments, and now…
Brazil, Chile, and Tanzania.
• We will review a series of CXR case studies and discuss an approach to the
diagnoses at hand: ACUTE CHEST SYNDROME.
Visit our website
www.EMGuidewire.com
for a complete archive of chest x-ray presentations and much more!
Airway
Bones
Cardiac
Diaphragm
Effusion
Foreign body
Gastric
Hilum
It’s All About The Anatomy!
25-Year-Old With A
History Of Sickle Cell
Disease Presents
With Severe Back
And Bilateral Leg
Pain.
Hospital Day 1
Afebrile And Vital
Signs Are Normal.
What Is Your Interpretation?
25-Year-Old With A
History Of Sickle Cell
Disease Presents
With Severe Back
And Bilateral Leg
Pain.
Hospital Day 3
Now Febrile,
Dyspneic,
Tachypneic And
Tachycardic.
25-Year-Old With A
History Of Sickle Cell
Disease Presents
With Severe Back
And Bilateral Leg
Pain.
Hospital Day 3
Now Febrile,
Dyspneic,
Tachypneic And
Tachycardic.
Acute Chest Syndrome
25-Year-Old With A
History Of Sickle Cell
Disease Presents
With Severe Back
And Bilateral Leg
Pain.
Hospital Day 3
Now Febrile,
Dyspneic,
Tachypneic And
Tachycardic.
Acute Chest Syndrome
25-Year-Old With A
History Of Sickle Cell
Disease Presents
With Severe Back
And Bilateral Leg
Pain.
Hospital Day 3
Now Febrile,
Dyspneic,
Tachypneic And
Tachycardic.
Acute Chest Syndrome
Side Notes:
Recall that the CXR
looked normal on
initial presentation.
This is the case for
≈50% of hospitalized
SCD patients who go
on to develop ACS.
Also, recall that
resolution of CXR
findings may lag
behind clinical
improvement.
25-Year-Old With A
History Of Sickle Cell
Disease Presents
With Severe Back
And Bilateral Leg
Pain.
Hospital Day 5
Clinically Improved
After Exchange
Transfusion.
Acute Chest Syndrome
25-Year-Old With A
History Of Sickle Cell
Disease Develops
Acute Chest
Syndrome.
Follow-Up CXR One
Month Later.
35-Year-Old With A
History Of Sickle Cell
Disease presents
Admitted For
Refractory Bilateral
Leg Pain.
CXR On Admission.
35-Year-Old With A
History Of Sickle Cell
Disease presents
Admitted For
Refractory Bilateral
Leg Pain.
Worsening Dyspnea
Three Days Later.
What Is Your Interpretation?
35-Year-Old With A
History Of Sickle Cell
Disease presents
Admitted For
Refractory Bilateral
Leg Pain.
Worsening Dyspnea
Three Days Later.
Acute Chest Syndrome
35-year-old with a history of Sickle Cell Disease Develops Acute Chest Syndrome.
35-Year-Old With A
History Of Sickle Cell
Disease Hospitalized
For Acute Chest
Syndrome.
Follow-Up CXR
Three Months Later.
Acute Chest Syndrome
 Defined as a new pulmonary infiltrate consistent with consolidation
[not atelectasis] of at least one lung segment.
 Usually accompanied by chest pain, cough, fever, and wheezing.
 The most common cause of ICU admission and premature death in
patients with sickle cell disease.
Gladwin M. New England Journal of Medicine 2008; 359:2254-65.
Acute Chest Syndrome
Three proposed mechanisms:
 Pulmonary infection
 Bronchoalveolar lavage demonstrates bacterial and/or viral pathogens in 54% of patients
with ACS.
 Embolization of bone marrow fat
 Associated with pain crisis of multiple bones, particularly the lumbar spine, femurs and the
pelvis.
 Pulmonary intravascular sickling and infarction
 In a small percentage of patients with ACS, wedge-shaped pulmonary infarcts may develop.
Gladwin M. New England Journal of Medicine 2008; 359:2254-65.
National Acute Chest Syndrome Study Group
 538 patients from 20 centers - the largest case series to date
 Results provide insights into the clinical presentations and outcomes of
hospitalized patients with ACS
49% of patients initially presented in pain crisis without signs of ACS!
Vichinsky EP. New England Journal of Medicine 2000; 342:1855-65.
National Acute Chest Syndrome Study Group
 Manifestations: worsening hypoxia, decreased hemoglobin levels, and
progressive, multi-lobar pulmonary infiltrates
 The mean hospital length of stay was 10.5 days [vs. 3 days w/o ACS]
 30% required mechanical ventilation and overall mortality was 3%
Vichinsky EP. New England Journal of Medicine 2000; 342:1855-65.
Infection1,2 33%
Pulmonary Infarction 33%
Pulmonary fat emboli 16%
1Pathogens identified using bronchoalveolar lavage
2Chlamydophilia, Mycoplasma pneumoniae & Respiratory Syncytial Virus the most common pathogens
Acute Chest Syndrome
ED Treatment Essentials:
 Antibiotics to cover both typical & atypical pathogens
 Supportive respiratory care
 A transfusion strategy based on goals and severity
Gladwin M. New England Journal of Medicine 2008; 359:2254-65.
Goal Target
Increase oxygen carrying capacity Hgb ≥10 grams
Manage vaso-occlusive complications HbS <30%
Both As above
Acute Chest Syndrome
ED Transfusion Caveats:
 If the patient worsens, start simple transfusion early
 2-4 units of PRBC on the way to the ICU
 Ramping up exchange transfusion takes time… don’t delay simple
transfusion waiting for this
American Society of Hematology
Hematology 2016
American Society of Hematology
Hematology 2016
RCE = Red Cell Exchange
American Society of Hematology
Hematology 2016
If you have interesting cases of Acute Chest Syndrome, we invite you
to send a set of digital PDF images and a brief descriptive clinical history to:
michael.gibbs@atriumhealth.org
Your de-identified case(s) will be posted on our education website and you
and your institution will be recognized!

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EMGuideWire's Radiology Reading Room: Acute Chest Syndrome

  • 1. Acute Chest Syndrome Gabriela Rivera Camacho, MD & Mark Kastner, MD Departments of Emergency Medicine & Hematology Carolinas Medical Center & Levine Cancer Institute Charlotte, North Carolina Michael Gibbs, MD Chest X-Ray Mastery Project™ Lead Editor Ify Osunkwo, MD Padmaja Veeramreddy, MD Guest Editors
  • 2. Disclosures  This ongoing chest X-ray interpretation series is proudly sponsored by the Emergency Medicine Residency Program at Carolinas Medical Center.  The goal is to promote widespread mastery of CXR interpretation.  There is no personal health information [PHI] within, and all ages have been changed to protect patient confidentiality.
  • 3. Process • Many of the provided clinical cases and presentations are then shared with all contributors on our departmental educational website. • Contributors from many Carolinas Medical Center departments, and now… Brazil, Chile, and Tanzania. • We will review a series of CXR case studies and discuss an approach to the diagnoses at hand: ACUTE CHEST SYNDROME.
  • 4. Visit our website www.EMGuidewire.com for a complete archive of chest x-ray presentations and much more!
  • 6. It’s All About The Anatomy!
  • 7. 25-Year-Old With A History Of Sickle Cell Disease Presents With Severe Back And Bilateral Leg Pain. Hospital Day 1 Afebrile And Vital Signs Are Normal.
  • 8. What Is Your Interpretation? 25-Year-Old With A History Of Sickle Cell Disease Presents With Severe Back And Bilateral Leg Pain. Hospital Day 3 Now Febrile, Dyspneic, Tachypneic And Tachycardic.
  • 9. 25-Year-Old With A History Of Sickle Cell Disease Presents With Severe Back And Bilateral Leg Pain. Hospital Day 3 Now Febrile, Dyspneic, Tachypneic And Tachycardic. Acute Chest Syndrome
  • 10. 25-Year-Old With A History Of Sickle Cell Disease Presents With Severe Back And Bilateral Leg Pain. Hospital Day 3 Now Febrile, Dyspneic, Tachypneic And Tachycardic. Acute Chest Syndrome
  • 11. 25-Year-Old With A History Of Sickle Cell Disease Presents With Severe Back And Bilateral Leg Pain. Hospital Day 3 Now Febrile, Dyspneic, Tachypneic And Tachycardic. Acute Chest Syndrome
  • 12. Side Notes: Recall that the CXR looked normal on initial presentation. This is the case for ≈50% of hospitalized SCD patients who go on to develop ACS. Also, recall that resolution of CXR findings may lag behind clinical improvement. 25-Year-Old With A History Of Sickle Cell Disease Presents With Severe Back And Bilateral Leg Pain. Hospital Day 5 Clinically Improved After Exchange Transfusion. Acute Chest Syndrome
  • 13. 25-Year-Old With A History Of Sickle Cell Disease Develops Acute Chest Syndrome. Follow-Up CXR One Month Later.
  • 14. 35-Year-Old With A History Of Sickle Cell Disease presents Admitted For Refractory Bilateral Leg Pain. CXR On Admission.
  • 15. 35-Year-Old With A History Of Sickle Cell Disease presents Admitted For Refractory Bilateral Leg Pain. Worsening Dyspnea Three Days Later. What Is Your Interpretation?
  • 16. 35-Year-Old With A History Of Sickle Cell Disease presents Admitted For Refractory Bilateral Leg Pain. Worsening Dyspnea Three Days Later. Acute Chest Syndrome
  • 17. 35-year-old with a history of Sickle Cell Disease Develops Acute Chest Syndrome.
  • 18. 35-Year-Old With A History Of Sickle Cell Disease Hospitalized For Acute Chest Syndrome. Follow-Up CXR Three Months Later.
  • 19.
  • 20. Acute Chest Syndrome  Defined as a new pulmonary infiltrate consistent with consolidation [not atelectasis] of at least one lung segment.  Usually accompanied by chest pain, cough, fever, and wheezing.  The most common cause of ICU admission and premature death in patients with sickle cell disease. Gladwin M. New England Journal of Medicine 2008; 359:2254-65.
  • 21. Acute Chest Syndrome Three proposed mechanisms:  Pulmonary infection  Bronchoalveolar lavage demonstrates bacterial and/or viral pathogens in 54% of patients with ACS.  Embolization of bone marrow fat  Associated with pain crisis of multiple bones, particularly the lumbar spine, femurs and the pelvis.  Pulmonary intravascular sickling and infarction  In a small percentage of patients with ACS, wedge-shaped pulmonary infarcts may develop. Gladwin M. New England Journal of Medicine 2008; 359:2254-65.
  • 22.
  • 23. National Acute Chest Syndrome Study Group  538 patients from 20 centers - the largest case series to date  Results provide insights into the clinical presentations and outcomes of hospitalized patients with ACS 49% of patients initially presented in pain crisis without signs of ACS! Vichinsky EP. New England Journal of Medicine 2000; 342:1855-65.
  • 24. National Acute Chest Syndrome Study Group  Manifestations: worsening hypoxia, decreased hemoglobin levels, and progressive, multi-lobar pulmonary infiltrates  The mean hospital length of stay was 10.5 days [vs. 3 days w/o ACS]  30% required mechanical ventilation and overall mortality was 3% Vichinsky EP. New England Journal of Medicine 2000; 342:1855-65. Infection1,2 33% Pulmonary Infarction 33% Pulmonary fat emboli 16% 1Pathogens identified using bronchoalveolar lavage 2Chlamydophilia, Mycoplasma pneumoniae & Respiratory Syncytial Virus the most common pathogens
  • 25. Acute Chest Syndrome ED Treatment Essentials:  Antibiotics to cover both typical & atypical pathogens  Supportive respiratory care  A transfusion strategy based on goals and severity Gladwin M. New England Journal of Medicine 2008; 359:2254-65. Goal Target Increase oxygen carrying capacity Hgb ≥10 grams Manage vaso-occlusive complications HbS <30% Both As above
  • 26. Acute Chest Syndrome ED Transfusion Caveats:  If the patient worsens, start simple transfusion early  2-4 units of PRBC on the way to the ICU  Ramping up exchange transfusion takes time… don’t delay simple transfusion waiting for this
  • 27. American Society of Hematology Hematology 2016
  • 28. American Society of Hematology Hematology 2016
  • 29. RCE = Red Cell Exchange American Society of Hematology Hematology 2016
  • 30. If you have interesting cases of Acute Chest Syndrome, we invite you to send a set of digital PDF images and a brief descriptive clinical history to: michael.gibbs@atriumhealth.org Your de-identified case(s) will be posted on our education website and you and your institution will be recognized!