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Key Features

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Essentials of Diagnosis
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  • Persistent symptoms of airway obstruction 8 weeks after the resolution of a lower respiratory tract infection

  • Chest CT shows a mosaic pattern of hyperinflation and vascular attenuation

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General Considerations
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  • Rare chronic obstructive lung disease

  • Characterized by complete obliteration of the small airways following a severe insult

  • Most common form in children is postinfectious, following a lower airway tract infection with adenovirus; influenza, rubeola, Bordetella, and Mycoplasma are also implicated

  • Other causes include

    • Connective tissue diseases

    • Chronic aspiration

    • Stevens-Johnson syndrome

    • Posttransplantation (lung or bone marrow)

    • Inhalational injury

  • Many cases of bronchiolitis obliterans are idiopathic

  • Mechanical ventilation for severe adenoviral respiratory infection is a strong risk factor

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Clinical Findings

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Symptoms and Signs
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  • Dyspnea

  • Cough

  • Exercise intolerance

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Differential Diagnosis
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  • Poorly treated asthma

  • Cystic fibrosis

  • Bronchopulmonary dysplasia

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Diagnosis

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  • Chest radiograph abnormalities include evidence of heterogeneous air trapping and airway wall thickening

  • Classic findings on high-resolution chest CT include

    • Mosaic perfusion pattern

    • Vascular attenuation

    • Central bronchiectasis

  • Ventilation-perfusion scans

    • May show pattern of mismatch

    • Not typically required for diagnosis

  • Pulmonary angiograms reveal decreased vasculature in involved lung

  • Bronchograms show marked pruning of the bronchial tree

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Treatment

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  • Supportive care

    • Supplemental oxygen for hypoxemia

    • Routine vaccination

    • Avoidance of environmental irritant exposure

    • Exercise

    • Nutritional support

  • Inhaled bronchodilators (β-agonists and anticholinergic) may reverse airway obstruction if the disease has a reactive component

  • Systemic corticosteroids

    • May help reverse the obstruction or prevent ongoing damage

    • Monthly pulse-dose corticosteroids will reduce the risk of adverse effects associated with long-term corticosteroids.

  • Antibiotics should be used as indicated for pneumonia

  • Azithromycin has been shown to have therapeutic benefit in bronchiolitis obliterans syndrome after lung transplantation

  • Lung transplant may be an option for patients with severe, progressive disease

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Outcome

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Complications
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  • Persistent airway obstruction

  • Recurrent wheezing

  • Bronchiectasis

  • Chronic atelectasis

  • Recurrent pneumonia

  • Unilateral hyperlucent lung syndrome

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Prognosis
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  • Depends in part on the underlying cause as well as the age of onset

  • Postinfectious bronchiolitis obliterans tends to be nonprogressive with low mortality and the possibility of slow improvement

  • Bronchiolitis obliterans resulting from transplantation or Stevens-Johnson syndrome may have a rapidly progressive course leading to death or need for lung transplantation

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References

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Colom  AJ  et al: Pulmonary function of a paediatric cohort of patients with postinfectious bronchiolitis obliterans. A long term follow-up. Thorax 2015 Feb;70(2):169–174
[PubMed: 25388479] .
CrossRef
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Li  YN  et al: Post-infectious bronchiolitis obliterans in children: a review of 42 cases. BMC Pediatr 2014 Sep 25;14:238
[PubMed: 25252824] .
CrossRef
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Teixeira  MF  et al: Acute bronchodilator responsiveness to tiotropium in postinfectious bronchiolitis obliterans in children. Chest 2013 Sep;144(3):974–980 ...

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