Acute Chest Syndrome

Diagnose acute chest syndrome (ACS) when a patient with sickle cell disease has a new pulmonary infiltrate involving at least one lung segment, excluding atelectasis, plus at least one compatible feature: chest pain, fever above 38.5°C, tachypnea, wheeze, or cough. The clinical-radiographic construct overlaps with pneumonia; therefore, obtain chest imaging in every patient with sickle cell disease who presents with respiratory symptoms. PubMed+1PubMedEssentials of PulmonologyPubMedAcute Chest Syndrome - StatPearls - NCBI Bookshelf

Treat new or increasing oxygen requirement, respiratory distress, hypoxemia, hypercapnia, extensive or progressive infiltrates, or declining clinical status as escalation signals. ACS can rapidly evolve into acute hypoxemic respiratory failure, multiorgan failure, and death; it is a leading cause of death in sickle cell disease. PubMed+2PubMedDiagnostic Test Accuracy of Lung Ultrasound for Acute Chest Syndrome in Sickle Cell Disease: A Systematic Review and Meta-analysis - PMCPubMedAcute Chest Syndrome - StatPearls - NCBI BookshelfPubMedSickle Cell Crisis - StatPearls - NCBI Bookshelf

Recognize differing dominant patterns by age and context. Children more often have fever, cough, wheeze, and increased work of breathing in infectious presentations, whereas adults may present with chest pain, dyspnea, extremity pain, or concurrent vaso-occlusive disease. ACS may develop during hospitalization for pain, after surgery, or after marrow infarction with fat embolism. ScienceDirect+2ScienceDirectHow I treat acute chest syndrome in children with sickle cell disease - ScienceDirectPubMedEssentials of PulmonologyPubMedAcute Chest Syndrome - StatPearls - NCBI Bookshelf

Obtain chest radiography, CBC with differential, oxygen-saturation assessment or arterial blood gas testing, standard chemistry testing, and blood group and screen in suspected ACS. A chest radiograph remains the reference imaging study for identifying the requisite new infiltrate, but an initially unrevealing film should not override an evolving clinical syndrome. PubMed+1PubMedSickle Cell Disease and Acute Chest Syndrome: Epidemiology, Diagnosis, Management, OutcomesPubMedAcute Chest Syndrome

Use arterial blood gas testing when oxygenation or ventilation is uncertain or worsening. A PaO2 below 60 mm Hg is a reported hypoxemia marker in ACS; rising carbon dioxide or respiratory acidosis supports escalation to noninvasive or invasive ventilatory support. ScienceDirect+1ScienceDirectA Survey of the Management of Acute Chest Syndrome in Children with Sickle Cell Disease in Centres in UK and Western Europe - ScienceDirectPubMedAcute Chest Syndrome

Order blood cultures, sputum bacterial cultures, nasopharyngeal or sputum viral testing, and other infection-directed studies when presentation and local epidemiology warrant them. These tests should refine etiologic management but should not delay empiric treatment for ACS. PubMed+1PubMedSickle Cell Disease and Acute Chest Syndrome: Epidemiology, Diagnosis, Management, OutcomesPubMedAcute Chest Syndrome

Lung ultrasound is a useful point-of-care adjunct when a rapid bedside assessment is needed. In a meta-analysis of six studies involving 625 patients, 97% aged 21 years or younger, pooled sensitivity was 0.92 and specificity was 0.89; applicability to adults remains less certain. PubMedPubMedDiagnostic Test Accuracy of Lung Ultrasound for Acute Chest Syndrome in Sickle Cell Disease: A Systematic Review and Meta-analysis - PMC

Administer supplemental oxygen for impaired gas exchange and titrate respiratory support to clinical severity. Noninvasive support such as CPAP may be used when oxygenation worsens, while intubation and invasive mechanical ventilation are indicated when respiratory dysfunction cannot be supported noninvasively. PubMedPubMedEssentials of Pulmonology In pediatric critical-care practice, worsening hypoxia and hypercapnia with respiratory acidosis are common triggers for noninvasive ventilation. ScienceDirect+1ScienceDirectA Survey of the Management of Acute Chest Syndrome in Children with Sickle Cell Disease in Centres in UK and Western Europe - ScienceDirectScienceDirectTherapeutic approach to pediatric patients with acute chest syndrome: national multicenter survey of non invasive ventilation (NIV) and transfusion - ScienceDirect

Use hydration cautiously rather than aggressively. ACS management includes modest or judicious fluid administration, because excess fluid can worsen pulmonary gas exchange in an acute lung injury syndrome. ScienceDirect+2ScienceDirectHow I treat acute chest syndrome in children with sickle cell disease - ScienceDirectPubMedAcute Chest SyndromePubMedSickle Cell Crisis - StatPearls - NCBI Bookshelf Provide analgesia promptly enough to permit deep breathing and coughing, but monitor closely for excessive opioid sedation and hypoventilation. PubMed+1PubMedAcute Chest SyndromePubMedSickle Cell Crisis - StatPearls - NCBI Bookshelf

Initiate empiric antibiotics because infection is a frequent precipitant and ACS is clinically difficult to distinguish from pneumonia. Macrolide antibiotics are included in pediatric ACS management recommendations; obtain indicated cultures before antibiotics when feasible without delaying therapy. ScienceDirect+2ScienceDirectHow I treat acute chest syndrome in children with sickle cell disease - ScienceDirectPubMedSickle Cell Disease and Acute Chest Syndrome: Epidemiology, Diagnosis, Management, OutcomesPubMedAcute Chest Syndrome

Use incentive spirometry and consider chest physiotherapy as lung-expansion measures, particularly in pain-associated hypoventilation or hospitalized vaso-occlusive episodes. Bronchodilators may be useful when wheeze or obstructive airway disease is present; routine use for every patient is not established by the cited evidence. ScienceDirect+1ScienceDirectA Survey of the Management of Acute Chest Syndrome in Children with Sickle Cell Disease in Centres in UK and Western Europe - ScienceDirectPubMedEssentials of Pulmonology

Consider simple transfusion when worsening anemia accompanies respiratory symptoms. This approach is used to improve oxygen-carrying capacity and reduce the proportion of circulating sickled erythrocytes, but the patient’s respiratory trajectory is more important than hemoglobin alone. ScienceDirect+1ScienceDirectHow I treat acute chest syndrome in children with sickle cell disease - ScienceDirectScienceDirectA Survey of the Management of Acute Chest Syndrome in Children with Sickle Cell Disease in Centres in UK and Western Europe - ScienceDirect

Consider partial exchange transfusion when respiratory status deteriorates despite only mild anemia, specifically when hemoglobin is greater than 9 g/dL in the cited pediatric treatment approach. This avoids excessive post-transfusion hemoglobin increase while reducing sickle hemoglobin exposure in progressive disease. ScienceDirectScienceDirectHow I treat acute chest syndrome in children with sickle cell disease - ScienceDirect

Involve critical care and hematology urgently for worsening hypoxemia, hypercapnia, respiratory acidosis, escalating noninvasive support, or need for invasive ventilation. Severe ACS has historically been managed with transfusion or exchange transfusion, although prospective comparative trials of these strategies are lacking. ScienceDirect+1ScienceDirectA Survey of the Management of Acute Chest Syndrome in Children with Sickle Cell Disease in Centres in UK and Western Europe - ScienceDirectScienceDirectTherapeutic approach to pediatric patients with acute chest syndrome: national multicenter survey of non invasive ventilation (NIV) and transfusion - ScienceDirect

Evaluate for infection, pulmonary fat embolism after marrow infarction, pulmonary infarction, perioperative stress, and pain-related hypoventilation. These mechanisms may coexist and should guide targeted testing and prevention rather than delay syndrome-directed management. ScienceDirect+3ScienceDirectHow I treat acute chest syndrome in children with sickle cell disease - ScienceDirectPubMedEssentials of PulmonologyPubMedDiagnostic Test Accuracy of Lung Ultrasound for Acute Chest Syndrome in Sickle Cell Disease: A Systematic Review and Meta-analysis - PMCPubMedSickle Cell Crisis - StatPearls - NCBI Bookshelf

After recovery, discuss hydroxyurea for recurrent ACS prevention. Hydroxyurea has reduced recurrent ACS episodes in children and adults, and chronic transfusion therapy has also been used to prevent recurrence in selected patients. Wolters Kluwer+1Wolters KluwerStandard management of sickle cell disease complications ☆ : Hematology/Oncology and Stem Cell TherapyScienceDirectHow I treat acute chest syndrome in children with sickle cell disease - ScienceDirect

For patients anticipating surgery, identify prior ACS, chronic lung disease, recent clustering of pulmonary complications, pregnancy, increasing age, and procedural invasiveness as features associated with greater perioperative pulmonary risk. Obtain a recent chest radiograph for baseline comparison when postoperative imaging may be needed, and assess for obstructive or restrictive lung disease when clinical history suggests it. PubMedPubMedEssentials of Pulmonology