A 7 1/2—year-old girl presents with breathlessness and fever for 6—7 days. She has non-productive cough for 6 months. XRay chest shows hyperlucency in the lungs and pulmonary function tests shows obstructive pattern. The most probable diagnosis will be
A. Lobar emphysema
B. Bronchiolitis obliterans
C. Follicular bronchitis
D. Pulmonary alveolar microlithiasis
Answer B (Bronchiolitis obliterans)
Bronchiolitis obliterans: It is a rare, chronic lung disease of the bronchioles and smaller airways.
Causes: It can occur after respiratory infections-viral: adenovirus, mycoplasma, etc.; Bacterial:
pertussis; connective tissue disease, SLE, etc; after lung and bone marrow transplantation.
• After initial insult, inflammation affecting terminal bronchioles, respiratory bronchioles and alveolar ducts, leads
to obliteration of airway lumen, that can be partial or complete.
• Partial or complete obstruction of airway lumen may result in airtrapping or atelectasis.
Clinical features: Cough, fever, cyanosis and respiratory distress followed by initial improvement, can be easily
confused with pneumonia, Bronchitis. Progression of disease leads to increasing dyspnea, cough, sputum
production and wheezing. CXR: May demonstrate hyperlucency and Patchy infiltrates. Pulmonary function test
(PFT): Typically show signs of airway obstruction. CT Scan chest: Patchy areas of hyperlucency and
bronchiectasis.
Treatment: No definitive treatment. Corticosteroids may be beneficial.
Follicular bronchitis: This is lymphoproliferative lung disorder characterized by the presence of lymphoid
follicles coursing alongside the airways (bronchi or bronchioles).
Pulmonary alveolar microlithiasis: characterized by the formation of lamellar concretions of calcium phosphate
or “microliths” within the alveoli.
Congenital lobar emphysema (CLE): Congenital deficiency of the bronchial cartilage, external compression by
aberrant vessels, bronchial stenosis, redundant bronchial mucosal flaps, and kinking of the bronchus caused by
hemiation into the mediastinum have been described as leading to bronchial obstruction and subsequent CLE.
