From the Guidelines
Pulmonary sequestration is a rare congenital lung malformation that is often associated with other congenital abnormalities, such as Williams Campbell syndrome and Mounier Kuhn syndrome, as noted in the British Thoracic Society guideline for bronchiectasis in adults 1. The diagnosis of pulmonary sequestration is typically made based on characteristic features on CT scanning of the chest, and it is essential to identify the aberrant blood supply to the sequestered lung tissue. Key points for understanding pulmonary sequestration:
- It is a congenital lung malformation that can present in adulthood, although it is rare
- It is often associated with other congenital abnormalities, such as Williams Campbell syndrome and Mounier Kuhn syndrome
- Diagnosis is typically made based on CT scanning of the chest
- The sequestered lung tissue is non-functional and prone to recurrent infections, and there is a risk of hemoptysis due to abnormal blood supply In the context of pulmonary sequestration, it is essential to consider the potential for complications, such as recurrent infections and hemoptysis, and to evaluate the need for surgical consultation for long-term management, as noted in the clinical practice guidelines for the management of community-acquired pneumonia in infants and children older than 3 months of age 1. The management of pulmonary sequestration should prioritize preventing complications and improving quality of life, and surgical resection may be necessary in some cases to achieve this goal. Key considerations for management:
- Diagnosis: Confirm with CT angiography to identify the aberrant blood supply
- Surgical consultation: Evaluate the need for surgical resection to prevent complications and improve quality of life
- Post-operative care: Standard post-thoracotomy care, including pain management and respiratory physiotherapy, if surgical resection is performed.
From the Research
Definition of Pulmonary Sequestration
- Pulmonary sequestration (PS) is a rare congenital anomaly that accounts for 1% to 6% of all pulmonary malformations at birth 2.
- It is characterized by a focal area of pulmonary tissue that does not have direct communication with the tracheobronchial tree and does not get blood supply from the pulmonary circulation 2.
- PS is a rare congenital lung malformation where extrapulmonary lung tissue receives systemic blood supply from an anomalous branch directly from the thoracic or abdominal aorta 3.
Clinical Features of Pulmonary Sequestration
- PS can present as a solitary nodule or mass, cystic lesion, consolidation, or an air-fluid level 2.
- Symptoms of PS include recurrent pulmonary infections, chest pain, and hemoptysis 2, 4.
- The period between symptom onset and diagnosis is typically more than five years 2.
Diagnosis of Pulmonary Sequestration
- Digital subtraction angiography is considered the gold standard for imaging, but computed tomography angiogram (CTA) is now preferred because it allows for clear visualization of lung parenchyma and vascular assisting in surgical planning 2.
- CTA can demonstrate a prominent tubular vessel, which shows less enhancement than the opacified pulmonary artery and pulmonary veins, suggestive of an abnormal vascular supply for the PS 2.
Management of Pulmonary Sequestration
- Management of intralobar sequestration is curative surgical excision 2, 4.
- Both video-assisted thoracoscopic surgery (VATS) and posterolateral thoracotomy are viable options for resection 2.
- Asymptomatic individuals with extralobar sequestration (ELS) should undergo serial monitoring as non-operative management is appropriate 2.
- Symptomatic patients of the pulmonary sequestration should be treated by surgery to avoid the risk of death due to massive hemoptysis 4.