Intralobar Pulmonary Sequestration Presentation
Intralobar pulmonary sequestration most commonly presents with recurrent pneumonia, which corresponds to option (c) in the multiple choice question.
Clinical Presentation of Intralobar Pulmonary Sequestration
Intralobar pulmonary sequestration (ILPS) is characterized by the following clinical features:
- Recurrent pneumonia (most common presentation)
- History of chronic cough
- Intermittent fevers
- Symptoms that may persist despite multiple courses of antibiotics
The evidence strongly supports recurrent pneumonia as the predominant presentation, with 90% of patients in one study having a history of recurrent pulmonary infections 1. This pattern of recurrent infections in the same lower lobe should raise suspicion for an intralobar sequestration.
Pathophysiology and Location
Intralobar pulmonary sequestration is a congenital malformation with these key characteristics:
- Non-functioning embryonic lung tissue
- Abnormal systemic arterial supply (typically from the thoracic or abdominal aorta)
- No normal connection to the tracheobronchial tree
- Most commonly located in the lower lobes (60-90%)
- Left lower lobe is more frequently affected than right (70% vs 30%)
The abnormal connection and drainage predispose the sequestered lung tissue to infection, explaining the pattern of recurrent pneumonia 2, 3.
Diagnostic Considerations
When evaluating a patient with suspected ILPS:
- Chest radiographs are abnormal but often non-specific
- CT with IV contrast or CTA chest is the preferred imaging modality 4
- CT can identify the sequestered lung tissue and its abnormal vascular supply
- Thoracic arteriography can visualize the systemic arterial supply and help prevent surgical complications
The diagnosis is often delayed (mean delay of 1.5 years in one study) due to symptoms being attributed to common respiratory conditions 1. In patients over 50, the diagnosis may be even more challenging as ILPS is rare in this age group 3.
Other Potential Presentations
While recurrent pneumonia is the most common presentation, other symptoms may include:
Some patients (approximately 10%) may be asymptomatic with the condition discovered incidentally on imaging 1.
Management
Definitive treatment involves surgical resection:
- Lobectomy is the most common procedure
- Video-assisted thoracoscopic surgery (VATS) is a minimally invasive option
- Identification and careful management of the aberrant systemic artery is crucial to prevent catastrophic bleeding
Early diagnosis and surgical intervention are important to prevent complications from recurrent infections and potential hemoptysis 1, 2.
Key Takeaway
When evaluating patients with recurrent lower lobe pneumonia, particularly in the same location, maintain a high index of suspicion for intralobar pulmonary sequestration. Early diagnostic imaging with contrast-enhanced CT or CTA is warranted to identify this uncommon but clinically significant condition.