Key Findings
- Purpose
To describe a rare case of plastic bronchitis complicating acute chest syndrome (ACS) in a child with sickle cell disease (SCD) and highlight the importance of early recognition of this potentially fatal complication. - Population (Model)
Single-patient case report involving a 5-year-old boy with HbSS, moderate persistent asthma, and a history of recurrent vaso-occlusive crises, acute chest syndrome, splenic sequestration, and hydroxyurea therapy, who was hospitalized for a vaso-occlusive pain crisis and subsequently developed acute chest syndrome during the admission. - Headline Result
Despite treatment for ACS, including broad-spectrum antibiotics, high-flow nasal cannula support, and simple transfusion, the child experienced rapid clinical deterioration, cardiovascular collapse, and death on hospital day three. Autopsy demonstrated plastic bronchitis with fibrinous airway casts causing approximately 95% airway obstruction throughout both lungs, together with pathological findings consistent with ACS. - Why It Matters
Plastic bronchitis is a rare and likely underrecognized complication of ACS in SCD. This case illustrates how rapid progression from worsening respiratory symptoms to fatal airway obstruction can occur within hours. The authors emphasize maintaining a high index of suspicion in patients with worsening respiratory distress, frothy productive cough, progressive bilateral pulmonary infiltrates, or pleural effusions despite appropriate ACS-directed therapies such as antibiotics, oxygen support, and transfusion. Bronchoscopy may serve both diagnostic and therapeutic roles in selected patients.
- Evidence Gaps & Limitations
This report describes a single patient and cannot establish the frequency of plastic bronchitis or identify definitive risk factors. The pathophysiology remains incompletely understood, and evidence for optimal diagnostic strategies and treatment is limited. Larger studies are needed to better define risk factors, improve early recognition, and evaluate interventions for plastic bronchitis such as bronchoscopy, airway-clearance strategies, and topical mucolytics, alongside ACS-directed measures like exchange transfusion.
Source: Journal of Sickle Cell Disease- “Sudden death caused by plastic bronchitis as a complication of acute chest syndrome in a child with sickle cell disease”
Regulatory & Guideline Watch
Existing sickle cell disease guidelines focus on early recognition and aggressive management of acute chest syndrome but, to the authors’ knowledge, do not address plastic bronchitis specifically, reflecting its rarity. This case highlights the importance of considering uncommon airway complications when respiratory status deteriorates rapidly despite appropriate ACS-directed therapy and supports further research to inform future clinical guidance.