Acute chest syndrome: Difference between revisions
 
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==Background==
==Background==
*A leading cause of hospitalization and death in adults with sickle cell disease
[[File:Lung and diaphragm.jpg|thumb|Lobes of the lung with related anatomy.]]
[[File:Computed tomograph of pulmonary vessels.jpg|thumb|Pulmonary arterial tree anatomy.]]
*The leading cause of death in patients with HbSS in the United States
**HbSS individuals are at higher risk than HbSC, but acute chest may occur in both genotypes
*Occurs most commonly in the 2-4yr old age group and then declines with age
*Occurs most commonly in the 2-4yr old age group and then declines with age
*Due to pulmonary ischemia and infarction; complication of pneumonia
*Pathophysiology is due to deoxygenation of Hb, leading to sickling in the pulmonary vasculature, causing vaso-occlusionm, ischemia, and endothelial injury<ref>Friend A, Settelmeyer TP, Girzadas D. Acute Chest Syndrome. [Updated 2023 Feb 6]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441872/</ref>
**In pediatric population, ACS is more often triggered by asthma or viral/bacterial pulmonary infection, leading to local hypoxia
**In adult population, ACS is commonly associated with vaso-occlusive pain, which may lead to hypoventilation or long bone fat/marrow emboli
**However, a specific cause is not identified in a majority of cases


===Causes===
===Causes===
*Pulmonary infection
*Pulmonary infection
**[[C. pneumoniae]] and [[mycoplasma]] are most common organisms
**[[Chlamydophila pneumoniae]] and [[Mycoplasma pneumoniae]] are most common organisms
**May also be caused by viruses, [[H. flu]], [[klebsiella]], [[staph]]
**May also be caused by [[S. aureus]], [[H. influenzae]], [[Klebsiella]], and viruses
*[[Fat emboli]]
***Infection due to [[S. pneumoniae]] is now rare due to pneumococcal immunization and prophylactic penicillin therapy
**Lodge in pulmonary vasculature → additional ischemia
*[[Fat embolism|Fat emboli]]
**Suspect if symptoms start 2-3 days following acute pain crisis
**Can cause microvasculature occlusion in the pulmonary circulation, leading to bone marrow infarction
**Autopsies have shown bony slivers and marrow fat found in pulmonary vasculature of patients
*Rib infarction
*Rib infarction
*Overly aggressive IV hydration
*Overly aggressive [[IVF|IV hydration]]
*[[Vaso-occlusive pain crisis]]
*[[Vaso-occlusive pain crisis]]
*[[Asthma]]
*[[Asthma]]
*Iatrogenic
**Opioid analgesics can lead to hypoventilation


==Clinical Features==
==Clinical Features==
*[[Fever]] >38.5
*New infiltrate on chest x-ray PLUS one of the following signs or symptoms
*[[Cough]]
**[[Fever]] >38.5°C (101.3°F)
*[[Wheezing]]
**[[Cough]]
*[[Tachypnea]]
**[[Wheezing]]
*[[Chest pain]]
**[[Tachypnea]]
**[[Chest pain]]
**[[Hypoxemia]]
 
==Complications==
*[[Pulmonary Embolism]] (bone marrow, fat or thrombotic)
*[[Pneumonia]]
*[[CVA]]
*[[Sepsis]]


==Differential Diagnosis==
==Differential Diagnosis==
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==Evaluation==
==Evaluation==
[[File:Transfusion-related acute lung injury chest X-ray.gif|thumb|Chest X-ray of TRALI (left) compared to the same subject after resolution of symptoms (right).]]
===Work-Up===
===Work-Up===
*CBC
*CBC
*Retic count
*Retic count
*VBG / ABG
*[[VBG]] / [[ABG]]
*Blood culture /sputum cultures
*[[Blood culture]] /sputum cultures
*[[CXR]]
*[[CXR]]
*Type and screen/crossmatch


===Evaluation===
===Imaging===
*New infiltrate on CXR with at least one of the following:
*New infiltrate seen on chest x-ray
**Fever >38.5
**Chest x-ray findings may lag behind clinical features
**Cough
*[[Lung ultrasound]] to CXR or CT finding correlations<ref>Razazi et al. Bedside Lung Ultrasound During Acute Chest Syndrome in Sickle Cell Disease. Medicine (Baltimore). 2016 Feb; 95(7): e2553.</ref>
**Wheezing
**Consolidation seen as hyperechoic punctiform air bronchograms
**Tachypnea
**Ground-glass opacities seen as coalescent B lines
**[[Chest pain]]
**[[Pleural effusion]], defined as large if interpleural distance > 25 mm
*Note: CXR findings may lag behind the clinical features
*Consider CTPE for persistent hypoxia with unclear cause


==Management==
==Management==
#O2
*[[O2]]
#*Titrate to pulse oximetry >92%
**Titrate to pulse oximetry >95%
#Incentive Spirometer
**NNoninvasive or invasive positive pressure ventilation may be required in severe cases
#Hydration
*Hydration
#*Oral hydration preferred
**[[oral rehydration therapy|Oral hydration]] preferred
#*[[IV hydration]] with hypotonic fluid if patient unable to tolerate PO
**[[IV hydration]] with hypotonic fluid if patient unable to tolerate PO
#Analgesia
***Consider D5 + 1/2 Normal saline at 1-1.5x maintenance rate
#*Pulmonary toilet is important but avoid excessive sedation
**While dehydration can predispose sickling, overhydration can cause pulmonary edema that exacerbates sickling
#[[Bronchodilators]]
*[[Analgesia]]
#[[Antibiotics]]
**Opioids often required for adults and sometimes needed for pediatrics
#*3rd generation [[cephalosporin]] + [[macrolide]]
**However, attempt to prevent oversedation and hypoventilation, atelectasis
#[[Transfusion]] (leucocyte depleted)
*Incentive Spirometry
#*Consider transfusion to goal of hemoglobin 11 / hematocrit 30 for:
*[[Bronchodilators]]
#**O2 Sat <92% on room air
*[[Antibiotics]]
#**hematocrit 10-20% below patient's usual hematocrit or dropping hematocrit
**3rd generation [[cephalosporin]] + [[macrolide]]
#[[Exchange transfusion]]
*Simple [[Transfusion]] (leucocyte depleted)
#*Consider for:
**Consider [[pRBCs|transfusion]] to goal of hemoglobin 11 / hematocrit 30 for:
#**Progression of acute chest syndrome despite simple transfusion
***O2 Sat <92% on room air
#**Severe hypoxemia
***hematocrit/hemoglobin 10-20% below patient's usual values, or continuously dropping hematocrit/hemoglobin
#**Multi-lobar disease
*[[Exchange transfusion]]
#**Previous history of severe acute chest syndrome or cardiopulmonary disease
**Consider for:
***Progression of acute chest syndrome despite simple transfusion
***Severe hypoxemia
***Multi-lobar disease
***Previous history of severe acute chest syndrome or cardiopulmonary disease
**Can prevent the need for intubation


==Complications==
==Disposition==
*[[Pulmonary Embolism]] (bone marrow, fat or thrombotic)
*Admission
*[[Pneumonia]]
*Consider consultation to hematologist
*[[CVA]]
*[[Sepsis]]


==See Also==
==See Also==
*[[Sickle Cell Crisis]]
*[[Sickle Cell Crisis]]


==Video==
==External Links==
{{#widget:YouTube|id=pKxAsqj4GOE}}
*[https://emcrit.org/ibcc/sickle-chest/ IBCC Chapter on Acute Chest Syndrome]
 
==References==
==References==
 
<references/>
[[Category:Heme/Onc]]
[[Category:Heme/Onc]]
[[Category:Pulmonary]]

Latest revision as of 18:51, 24 April 2024

Background

Lobes of the lung with related anatomy.
Pulmonary arterial tree anatomy.
  • The leading cause of death in patients with HbSS in the United States
    • HbSS individuals are at higher risk than HbSC, but acute chest may occur in both genotypes
  • Occurs most commonly in the 2-4yr old age group and then declines with age
  • Pathophysiology is due to deoxygenation of Hb, leading to sickling in the pulmonary vasculature, causing vaso-occlusionm, ischemia, and endothelial injury[1]
    • In pediatric population, ACS is more often triggered by asthma or viral/bacterial pulmonary infection, leading to local hypoxia
    • In adult population, ACS is commonly associated with vaso-occlusive pain, which may lead to hypoventilation or long bone fat/marrow emboli
    • However, a specific cause is not identified in a majority of cases

Causes

Clinical Features

Complications

Differential Diagnosis

Sickle cell crisis

Evaluation

Chest X-ray of TRALI (left) compared to the same subject after resolution of symptoms (right).

Work-Up

Imaging

  • New infiltrate seen on chest x-ray
    • Chest x-ray findings may lag behind clinical features
  • Lung ultrasound to CXR or CT finding correlations[2]
    • Consolidation seen as hyperechoic punctiform air bronchograms
    • Ground-glass opacities seen as coalescent B lines
    • Pleural effusion, defined as large if interpleural distance > 25 mm
  • Consider CTPE for persistent hypoxia with unclear cause

Management

  • O2
    • Titrate to pulse oximetry >95%
    • NNoninvasive or invasive positive pressure ventilation may be required in severe cases
  • Hydration
    • Oral hydration preferred
    • IV hydration with hypotonic fluid if patient unable to tolerate PO
      • Consider D5 + 1/2 Normal saline at 1-1.5x maintenance rate
    • While dehydration can predispose sickling, overhydration can cause pulmonary edema that exacerbates sickling
  • Analgesia
    • Opioids often required for adults and sometimes needed for pediatrics
    • However, attempt to prevent oversedation and hypoventilation, atelectasis
  • Incentive Spirometry
  • Bronchodilators
  • Antibiotics
  • Simple Transfusion (leucocyte depleted)
    • Consider transfusion to goal of hemoglobin 11 / hematocrit 30 for:
      • O2 Sat <92% on room air
      • hematocrit/hemoglobin 10-20% below patient's usual values, or continuously dropping hematocrit/hemoglobin
  • Exchange transfusion
    • Consider for:
      • Progression of acute chest syndrome despite simple transfusion
      • Severe hypoxemia
      • Multi-lobar disease
      • Previous history of severe acute chest syndrome or cardiopulmonary disease
    • Can prevent the need for intubation

Disposition

  • Admission
  • Consider consultation to hematologist

See Also

External Links

References

  1. ↑ Friend A, Settelmeyer TP, Girzadas D. Acute Chest Syndrome. [Updated 2023 Feb 6]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK441872/
  2. ↑ Razazi et al. Bedside Lung Ultrasound During Acute Chest Syndrome in Sickle Cell Disease. Medicine (Baltimore). 2016 Feb; 95(7): e2553.
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