The role of timely intervention in middle lobe syndrome in children

Study objectives: Middle lobe syndrome (MLS) in children is characterized by a spectrum of clinical and radiographic presentations, from persistent or recurrent atelectasis to pneumonitis and bronchiectasis of the right middle lobe (RML) and/or lingula. This study was undertaken to evaluate the effect of early intervention, including fiberoptic bronchoscopy (FOB), in the development of bronchiectasis in MLS. Design: Children with atelectasis of the RML and/or lingula persisting for > 1 month or recurring two or more times despite conventional treatment underwent high-resolution CT (HRCT) scanning and FOB. Appropriate treatment and follow-up were provided, and the effect of the duration of symptoms on clinical outcome and the development of bronchiectasis was investigated. The patient cohort was retrospectively reviewed. Patients: We evaluated 55 children with MLS. The median age at diagnosis, duration of symptoms, and duration of clinical deterioration before diagnosis were 5.5 years (range, 3 months to 12 years), 14.5 months (range, 3 to 48 months), and 8 months (range, 3 to 36 months), respectively. Measurements and results: FOB revealed marked obstruction in two children (ie, a foreign body and an endobronchial tumor) and positive findings for a culture of BAL fluid in 49.1% of patients. The remaining 53 patients were followed up for a median duration of 24 months (range, 5 to 96 months). The clinical outcome was "cure" in 60.4% of patients, "improvement" in 32.1% of patients, and "no change" in the remaining patients. Bronchiectasis was documented prior to FOB by HRCT scan in 15 patients (27.3%). The duration of the deterioration of symptoms prior to presentation positively correlated with the development of bronchiectasis (p = 0.03) and an unfavorable clinical outcome (ie, improvement or no change) [p = 0.02]; a positive correlation was also found between the duration of symptoms and the development of bronchiectasis (p = 0.04). Conclusions: Timely medical intervention in patients with MLS that includes FOB with BAL prevents bronchiectasis that may be responsible for an ultimately unfavorable outcome.