Medical Care
Long-term follow-up of children with right middle lobe syndrome (RMLS) shows that most patients do not experience recurrent or persisting symptoms. This indicates that the first line of treatment in all cases is conservative medical management, except in cases involving neoplastic origin and those with bronchiectasis.
Chest physical therapy and postural drainage are the hallmarks of therapy. They may be combined with mucolytics such as nebulized sodium chloride (3% or 7%, based on the patient's age and tolerance), and or dornase alpha to help optimize airway clearance. In persistent or severe cases, especially those with bronchiectasis, patients may benefit from the use of high frequency chest wall oscillation devices.
Treat the asthmatic child with aggressive anti-inflammatory therapy such as inhaled steroids. Consider systemic steroids. Guidelines for the diagnosis and management of asthma have been established. [6]
Provide the patient with chest physical therapy and postural drainage. In unresponsive patients or patients who have a predisposition to airway colonization, an appropriate antibiotic, as determined by a bronchoalveolar lavage (BAL) culture, should be added to their regimen.
Patients with fungal infections (eg, histoplasmosis) or tuberculous infections who have hilar adenopathy and complete blockage of their right middle lobe should be treated aggressively. The addition of systemic corticosteroids may be necessary.
Surgical Care
Lobectomy
Lobectomy is indicated in cases of malignancy and bronchiectasis that are unresponsive to medical therapy.
Only perform lobectomy when right middle lobe syndrome is associated with systemic symptoms such as failure to thrive, persistent cough, and recurrent fever or when chronic infection threatens the remainder of the lung. The majority of patients with appropriate aggressive medical therapy will rarely require surgical intervention.
Bronchography
Avoid bronchography because of potential risks to the patient unless surgery is seriously considered; therefore, always explore high-resolution CT imaging as an alternative.
Consultations
Consultations may include a pediatric pulmonologist and/ or a pediatric infectious disease specialist.
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Posterioranterior chest radiograph demonstrating right middle lobe infiltrate in a 9-year-old male with a history of severe asthma. Note the blurred right heart border.
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Lateral view chest radiograph in a 9-year-old male with severe asthma showing a wedge-shaped density extending from the hilum anteriorly and inferiorly.
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High-resolution chest CT showing marked chronic changes and volume loss in the right middle lobe with diffuse traction bronchiectasis in 9-year-old male with a history of severe asthma and RML syndrome.
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Posteroanterior chest radiograph demonstrating right middle lobe collapse and infiltrate. Note blurred right heart border.
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Lateral view chest radiograph showing a wedge-shaped density extending from the hilum anteriorly and inferiorly.
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Chest CT scan showing extensive bronchiectasis of both medial and lateral segments of the right middle lobe.
