Treatment of Extralobar Pulmonary Sequestration
The treatment of choice for extralobar pulmonary sequestration is surgical excision, which should be performed as early as possible after diagnosis to prevent complications such as infection, hemorrhage, or malignant transformation. 1, 2
Understanding Extralobar Pulmonary Sequestration
- Extralobar pulmonary sequestration (EPS) is a rare congenital lung malformation characterized by non-functional lung tissue that is anatomically separate from the normal lung and typically receives its blood supply from systemic vessels 1
- Most commonly located between the diaphragm and left lower lobe (77.4% of cases), though they can also occur in the mediastinum, within the diaphragm, or rarely below the diaphragm 1, 2
- Unlike intralobar sequestrations, EPS has its own pleural covering and does not communicate with the normal tracheobronchial tree 1
- The arterial supply is typically from branches of the aorta, though rare cases with pulmonary arterial feeding vessels have been reported 3, 2
Diagnostic Evaluation
- Three-dimensional reconstruction computed tomography (CT) is essential to identify the abnormal blood vessels supplying the sequestration 3
- CT chest without contrast is appropriate for evaluating the lung parenchyma and pleural abnormalities 4
- Contrast-enhanced CT is indicated when vascular anomalies such as pulmonary sequestration are suspected, to clearly identify feeding vessels 4
- The typical radiologic finding is a homogeneous soft-tissue mass, usually in the lower hemithorax 1
- Radiologic diagnosis relies on identification of the systemic vascular supply, which historically required angiography but can now be demonstrated with ultrasound, CT, or MRI 1
Treatment Approach
- Surgical excision is the definitive treatment for extralobar pulmonary sequestration 1, 2
- Video-assisted thoracic surgery (VATS) is a useful and minimally invasive approach for resection of extralobar sequestration 3
- For abdominal extralobar sequestrations, laparoscopic excision may be appropriate 5
- Early surgical intervention is recommended to prevent complications, even in asymptomatic cases 2
- Careful identification and management of the anomalous feeding vessels is critical to prevent potentially fatal hemorrhage during surgery 2
Timing of Intervention
- For symptomatic patients (respiratory distress in newborns or infants), prompt surgical excision is indicated 1
- For asymptomatic patients discovered incidentally, elective surgical excision is still recommended due to the risk of later complications 1, 2
- In some pediatric cases, observation with follow-up imaging may be considered initially, but surgical excision is indicated if there is no evidence of regression 5
Potential Complications
- Infarction of extralobar pulmonary sequestration can occur after trauma, necessitating urgent surgical intervention 6
- Secondary changes such as tuberculosis or carcinoma have been reported in approximately 6 cases out of 133 extralobar sequestrations 2
- Operative mortality is primarily related to hemorrhage during operation due to inadequate control of the anomalous vessels 2